Term
|
Definition
| kinase target of rapamycin that controls cell size |
|
|
Term
|
Definition
| enriched with cholesterol, sphingolipds, and detergent resistant |
|
|
Term
|
Definition
| NOT a cilium, small, antenna, wound healing, , orientation in cell division, 9-2 or other confirmations |
|
|
Term
| 1st step in making steriods |
|
Definition
| done in the mitochondria as it takes in cholesterol |
|
|
Term
|
Definition
| steriod hormones and lipds, calcium, detox |
|
|
Term
| small structures of the nucleus |
|
Definition
|
|
Term
|
Definition
| rRNA, ribosomes, telomerase |
|
|
Term
|
Definition
break open with isotonic buffer,
centrifuge
purify, sucrose centrifugation
marker enzymes or microscope |
|
|
Term
|
Definition
whole cells, nuclei, cytoskeleton
mitochondria lysosomes, perioxisomes
microsomes, small vesicles, rebisomes, viruses, large molecules |
|
|
Term
|
Definition
| device to cut very slim area of tissue |
|
|
Term
| scanning vs transmission EM |
|
Definition
scanning is 3D but only sees surface
transmission is 2D but can see inside the cell |
|
|
Term
|
Definition
| cell lines start to die off |
|
|
Term
|
Definition
what light amplitudes get through
the wavelength change by density of the liquid it went through |
|
|
Term
|
Definition
normally right handed, 3.4 A between pairs, 34A to a turn
A - minimal water
Z - left handed |
|
|
Term
|
Definition
H2A, H2B, H3 and H4 make up the nucleosome(single rung), while octomer is these joining together (200 base pairs per nucleosome)
H1 (much smaller) binds DNA at edge of EACH nucleosome and form center ring, modified at tail |
|
|
Term
|
Definition
| histones replaced with protamines which are arginine rich and tightly package DNA |
|
|
Term
| What is the smallest chrosome? |
|
Definition
|
|
Term
| Different centromere positions |
|
Definition
metacentric - 1,2,3,19,20
submetacentric - 4,5,6,7,8,9,10,11,12
acrocentric - 13,14,15,21,22 |
|
|
Term
|
Definition
Dark - condense, hetero, A-T
Light - GC, euchomatin
(think that GC is already bound tightly, no room for stain and that CG signals a gene area and is therefore euchromatin) |
|
|
Term
|
Definition
A-1,2,3, meta (except A which is sub)
B- 4,5 sub
C- 6,7,8,9,,10,11,12 sub
D- 13,14,15 acc
E-16,17,18 sub
F - 19,20 meta
G- 21,22 acc |
|
|
Term
miscarriage in 8-11 wks
overall |
|
Definition
|
|
Term
| most chromosome abnormalities on mortality and morbidity |
|
Definition
3-15%
Exceptions: sexual stuff (hemaphrodite 25, ovarian def 65, pubertal development 27) |
|
|
Term
|
Definition
| denature DNA, insert DNA binding probe |
|
|
Term
|
Definition
| RNA that is transcribed but not translated that goes and shuts off that chromosomes |
|
|
Term
|
Definition
| region of similarity between X and Y, at distal P arms, recombination can happen there and one other spot at end of q rm |
|
|
Term
|
Definition
| arrestis diplotene (20 ws) until ovulation |
|
|
Term
|
Definition
| loss of a chromosomes happnens in M1 or M2 |
|
|
Term
|
Definition
| mismatch repair protein used to show recombination sites |
|
|
Term
| nondisjunction and recombination |
|
Definition
| more recombination organizeschromosomes together and makes less nondisjnunction |
|
|
Term
|
Definition
mitotic nondisjunction,
meiotic anaphase lag of a trisomy |
|
|
Term
|
Definition
| confined placental mosaicism |
|
|
Term
|
Definition
|
|
Term
| How many Down Sundrome babies abort spontaneously? |
|
Definition
66%
1/700 births
4% translocation |
|
|
Term
|
Definition
60% bad hearing
20 bad thyroid
5 ALL
Males are sterile, 40% of females ovulate
40 heart defects |
|
|
Term
|
Definition
Patau
1/5000
20% transloations
feeding problems, oral celfts,
95% die before 3
undescended testes, |
|
|
Term
|
Definition
Edward Syndrome
1/3000
95% aborted
4% translocations
heart defect, low birth weight, fingers that are clinched with 2 and 5 overlap
rocker bottom feet
85% die in first year |
|
|
Term
|
Definition
80% paternal chromosome lost
98% aborted
webbed neck (cause necks are hot and they are not)
space nipples
no secondary sex characteristics
ovarian failure |
|
|
Term
|
Definition
1/660 births
50% aborted
long arms,tall, bad sperm
15% mosaic
50/50 paternal or maternal |
|
|
Term
|
Definition
Tall, large teeth, normal fertility, learning disabilities
100% paternal |
|
|
Term
|
Definition
|
|
Term
triploids!
diandric
digynic |
|
Definition
dyandric - male - large fetus / placenta, do not survive frequently
digynic - female (women would go to gynocologist) growth retarded To not hurt mom!), survive up to a year
both: join digits 3 and 4, ossification of skull |
|
|
Term
|
Definition
| spontaneous abortions, 6% are chromosoml related among couples with 2 or more |
|
|
Term
|
Definition
| spontaneous abortions (trisomys are half this), 66% at less then 8 weeks, 40% for all 1st trimester, .5% liveborns |
|
|
Term
| meiotic nondisjunction errors mom or dad? |
|
Definition
|
|
Term
del
ins
inv
t
rep
rob
dup
mar
r
iso |
|
Definition
deletion
insertion
inversion
translocation
reciprocal translocation
robertsonian transoloation
duplications
marker
ring
isochromosomes |
|
|
Term
|
Definition
mental retardation and congenital malformations
1/7000
interstitial - 2 breaks and faulty repiair
terminal - break and loss of acentric frag
unequal crossing over
translocation/inversion parent crossing over |
|
|
Term
|
Definition
del5p or just (p15-pter)
15% translocation |
|
|
Term
|
Definition
unequal crossing over
abnormal segrgation in meosis of a balenced parent
less detrimental than deletions |
|
|
Term
|
Definition
| missing limbs, large developmental delay, , duplication in 3q |
|
|
Term
|
Definition
overgrowth disorder
duplication in distal 11p |
|
|
Term
|
Definition
| two copies from one parent and none from the other |
|
|
Term
|
Definition
| uniparental disomy or dynamic mosaiscism |
|
|
Term
| ring chromosome syundrome |
|
Definition
| 99% de novo, metal retardations, if inherited its maternal, partial monosomy |
|
|
Term
|
Definition
deletion of one arm and dubplication of the other
Causes
1) exchange with sister chromatid
2) pulled transverse in division
they are a partial monosomy and artial trisomy
seen in tumors! |
|
|
Term
|
Definition
1/4000 births
small and unidentifiable chromosomes, hard to see, mostly fish,
80% derived from acrocentrics
80% de novo and show maternal age effect |
|
|
Term
two trnaslocations types:
centric fusion
reciprocal |
|
Definition
robertsonian
between autosomes |
|
|
Term
reciprocal translocation
what percent are inherited?
what do they form at meisosis? |
|
Definition
70%
activate chancer genes,
quadrivalent
6 gamete possibilities (1 normal 1 balenced) |
|
|
Term
11;22 Reciprocal translocation
4;8 translocation |
|
Definition
| inherited, usually 47 chromosomes from 3:1 segregation, (extra is der22), 3% risk for male carriers, 5-7% for females |
|
|
Term
| robertsonian translocation |
|
Definition
joining of two accentric chromosomes
common 13,14 or 14,21
dicentric with one centromere suppressed (can be a problem)
carriers have fertlity problems, uniparental disomy children |
|
|
Term
|
Definition
20% of cases, 55% of parents are carriers
carrier parents only has 1% chance of having an affected child |
|
|
Term
|
Definition
4% unbalenced robersonian translocations
60% have carrier parent
25 mosiac
theoretical risk of liveobrn -33%, empirical 10% |
|
|
Term
|
Definition
90% of inversions are inheritied
peri - Involves centromere
para - (spanish for FOR FORGET about the centromere)
duplications and deletions of ends, not loops (means para creates bad centromere types and are lethal) |
|
|
Term
Structural rearrangments
paternal or maternal? |
|
Definition
PATERNAL!
expections: robertsonian (90%) materinal
Digeorde and Williams Beuren have no bias |
|
|
Term
| What types of amino acids are in cells? |
|
Definition
| L! L is twice in the word cell |
|
|
Term
|
Definition
| Those Ten Valuable Aminoacids Have Long Presrved Life In Man |
|
|
Term
| Nonpolar aliphatic R groups |
|
Definition
|
|
Term
|
Definition
Wash Your Foot!
(aromatic, smell)
absorb light |
|
|
Term
| amino acids with polar R groups |
|
Definition
| Queen Noor Could Stop Terrorsism |
|
|
Term
| Amino acids with positive charge |
|
Definition
|
|
Term
| negative charge amino acids |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
| affects activity, transient |
|
|
Term
|
Definition
| sugars, make hydrophillic |
|
|
Term
|
Definition
|
|
Term
|
Definition
| the nitrogen as that is made first by ribosome |
|
|
Term
| What kind of bonds for secondary structure? |
|
Definition
|
|
Term
|
Definition
3.6 amino acids per turn
NH and CO groups of the main chain
right handed
groups are pointed outwards |
|
|
Term
|
Definition
| much 'longer' between AA, antiparalell is longer |
|
|
Term
|
Definition
glycine and pro are good
glycine is small
proline does cis and can do tight turn |
|
|
Term
|
Definition
2 Cysteines coming together
tertiary structure |
|
|
Term
|
Definition
commonly recognized secondary structure
regions of a protein |
|
|
Term
|
Definition
| VERY similar in structure, completely different primary structure |
|
|
Term
| how does the structure of Hem change? |
|
Definition
| Fe pulls down histidine and puts it into line with 4 nitrogens, makes it into RELAXED (not tense state) |
|
|
Term
|
Definition
| glutamate is replaced by valine on B chains (negative to neutral) |
|
|
Term
| fibrous vs. globular proteins |
|
Definition
fibrous - long strands, shape and support, insoluable
globular - sphereical, enzymes, regulatory, soluable |
|
|
Term
|
Definition
| long, triple helix, Gly-Pro-4Hyp,strands are bond together, no bonds within a strand |
|
|
Term
|
Definition
replacement of Cys or Ser with for Gly
bad bones in babies |
|
|
Term
|
Definition
comes out after heat!
chaperones
uses ATP |
|
|
Term
|
Definition
| chaperonin, big barrel, use ATP |
|
|
Term
| Protein disulfide isomerase |
|
Definition
|
|
Term
| peptide prolyl cis-trans isomerase |
|
Definition
| shifts the prolines cis and trans |
|
|
Term
|
Definition
phsophoglycerides
sphingolipids
sterols |
|
|
Term
|
Definition
phosphotidylethanolamine
phosphatidylserine (neg charge)
phosphatidylcholine |
|
|
Term
| Which membrane molecule is a phospholipid but not a phosphoglyceride? |
|
Definition
|
|
Term
| which phospholipid should you not see on the surface of the molecule? |
|
Definition
| phosphatidylserine and its negative charge are a sign of apoptosis |
|
|
Term
|
Definition
| Glycosylphosphatidylinositol (GPI anchor) is a glycolipid that can be attached to the C-terminus of a protein during posttranslational modification. It is composed of a phosphatidylinositol group linked through a carbohydrate-containing linker |
|
|
Term
|
Definition
out to inner
inner to outer
either direction till at equilibrium |
|
|
Term
| rafts in membrane are made of what molecules? |
|
Definition
| sphingolipids and cholesterol clusters |
|
|
Term
|
Definition
| carbohydrates above the lipid layer outside of protein |
|
|
Term
| How do you make membrane more gel like? |
|
Definition
| saturated and low temperature |
|
|
Term
|
Definition
| groups different singals processes togetehr |
|
|
Term
|
Definition
| forms linke between actin junctional complexes to helpcells resist shear (beneath membrane), restricts diffusion and causes corralling |
|
|
Term
| Which are faster carriers or channels? |
|
Definition
|
|
Term
|
Definition
| both involve 2 things, its just whether they are going in the same or opposite directions |
|
|
Term
|
Definition
facillitated diffusion of Glucose into RBC
Has diffusion down gradient, saturabiltiy and specificity
glucose could leave through this just as easily! |
|
|
Term
| Chloride bicarbonate exchanger |
|
Definition
antiport for HC03- out and Cl- in to keep membrane potential the same
(HC03- leaves in tissue to pick upmore CO2), enters RBC in lungs to make more CO2 |
|
|
Term
| what are the two types of active transport? |
|
Definition
primary - atp energy moves molecule directly
secondary - potential set up and symporter used |
|
|
Term
|
Definition
3/2 pump tat sets up membrane potential, simply a primary active transporter
uses Enz |
|
|
Term
| Glucose transport in intestinal epithelial cells |
|
Definition
| secondary transport - glucose comes in in the symporter with 2Na+ that has had a gradient set up for it by NKATPase |
|
|
Term
|
Definition
| 4 identical monomers, allows passage of water |
|
|
Term
|
Definition
| ATP is the ligand that binds to the NBD and opens the channel, |
|
|
Term
|
Definition
| not directly bound to DNA |
|
|
Term
|
Definition
1 - most rRNA
2 - normal mRNA being made
3 - tRNA and some rRNA (3-T rhymes!) |
|
|
Term
|
Definition
| assessory proteins used to guide RNA polymerase to promoter |
|
|
Term
| What is the number of the carbon that goes from oxy to deoxy for DNA? |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
| microRNA binds to RNA sequences to silience them |
|
|
Term
|
Definition
| small interferring RNA, turn off gene expression by directing degradation og mRNA's and causing chromatin structure |
|
|
Term
| How does the cell know when to do poly-adenylation therein stopping trancription? |
|
Definition
|
|
Term
| Who plays a role in splicing? |
|
Definition
|
|
Term
| Which end goes through the nuclear pore first? |
|
Definition
| 5' end, dont forget the nuclear export receptor! |
|
|
Term
| Who degrades mRNA in the cytosol with siRNA? |
|
Definition
|
|
Term
| What eats up badly fold proteins? |
|
Definition
| proteosome, marked by ubiquitin tail |
|
|
Term
|
Definition
| Asp, in ER for surface or lysosome |
|
|
Term
|
Definition
| Ser,, cytosol or golgi, out of the cell |
|
|
Term
|
Definition
| activation changes the chromatin structure, modulation turns on transcription |
|
|
Term
insulator element
barrier sequence |
|
Definition
insulator element does not allow an enhancer to get to the genes past it
barrier sequence separates Eu and hetero chromatin |
|
|
Term
| mRNA localization mechanisms |
|
Definition
directed transport on cytoskeleton
random diffusion and trapping
degradation to make sure it is not in some areas |
|
|
Term
| Where do proteins without a signal end up? |
|
Definition
|
|
Term
| What if a signal sequence is kept on a protein after it enters the organelle? |
|
Definition
| maybe it is staying there or maybe it is going to leave and come back (retreval) |
|
|
Term
|
Definition
| nucleoporins, cdifferent types can be mutated, causing disease |
|
|
Term
|
Definition
| bring molecules in and out of the nuclear pore, they bind at FG repeats |
|
|
Term
|
Definition
Ran GDP in cytosol (made by Ran GAP)
Ran GTP in nucleus (made by Ran GEF)
Ran-GEF is bound to chromatin
A is closer to D
E is closer to T |
|
|
Term
|
Definition
Ran GDP Disassociates from receptors and they go back alone!
Ran GTP binds to receptors and brings them out
use the D in disassociates here also MAKES SENSE as GTP has the energy and therefore binds to use its energy! |
|
|
Term
|
Definition
TOM - mitochondria outer membrane complex
TIM - inner mitochondria membrane channe, 23 as Hsp70
SAM - helps proteins unfold and become a part of the outer membrane
OXA - insertion of inner membrane proteins that are in the mitochondria
TIM and TOM can be used at the same time |
|
|
Term
| What signal brings proteins into the mitochondria? |
|
Definition
| alpha helix membrane signal with positive charge attrated to the membranes negative charge on matrix side on inner membrane |
|
|
Term
| Pyruvate dehydrogenase deficiney - PDHE1a |
|
Definition
| bad targeting signal to mitochondria, |
|
|
Term
| atypical mitochondria disease with multisystem failure |
|
Definition
| defects in processing or mitochondrial chaperones |
|
|
Term
|
Definition
aquired defects in mitochondrial protein import
may be by ROS |
|
|
Term
|
Definition
it remains intact, unlike mitochondria signal which is degraded!
meets up ith Pex5 (cytosol),14 (membrane) |
|
|
Term
| X-linked adrenoleukodystropy (X-ALD) |
|
Definition
affects brain and adrenal cortex
mutation in peroxisomal ABC half transporter, so it can not import very long fatty acids |
|
|
Term
|
Definition
metabolism of lipds
drug detox
glycogen degradation
Ca fluxes |
|
|
Term
|
Definition
n linked glycosylation
hydroxylation of Pro, lys cleavase
folding and subunit association |
|
|
Term
|
Definition
| signal recognition particle, pauses translation when it sees the signal and brings ribosome to ER |
|
|
Term
|
Definition
| ER quality control, keep bad prteins from leaving the ER to the golgi |
|
|
Term
|
Definition
retrieval sequence to bring protein back to the ER from the golgi
binds to KDEL receptor in Golgiwhich is a membrane protein part of the retrieval vesical |
|
|
Term
|
Definition
N is in the ER
O is in the Golgi and goes OUT of the cell
(triple O, O, gOlgi, Out |
|
|
Term
What amino acid does N link occur on?
Does it matter which one? |
|
Definition
|
|
Term
|
Definition
checks protein, if still not folded correctly it adds a sugar so that the protein can hang out longer
needs mannose to be recognized by this protein |
|
|
Term
| calnexin and calreticulin |
|
Definition
| require Ca2+ to work, only check proteins with one glucose (must have lost others over time by glucosidases |
|
|
Term
| Where does tagging with ubiquitin occur in the cell? |
|
Definition
| cytosol after glycanase gets rid of all sugars and are translocator protein pushes protein and remaining sugar out of cell |
|
|
Term
|
Definition
ubiquitin proteasomal system
E1 activates ub
E2 binds it to specific proteinss
it is activated from signals by the ER |
|
|
Term
where are proteosomes located?
what are inside them? |
|
Definition
nucleus and cytosol
proteases! |
|
|
Term
| what fragments show up in alziehmers disease? |
|
Definition
AB fragments-amyloid plaques
neurofibrillary tangles |
|
|
Term
| When do we see agrregates? |
|
Definition
| there is a poorly folded protein too fast for proteosomes to eat it up! |
|
|
Term
| junvenile parkinson's disease |
|
Definition
|
|
Term
| How many membranes for autophagy? |
|
Definition
2!
Remember this with fuse with a lysosome! |
|
|
Term
|
Definition
| coat molecule from plasma membrane and between endosomes aND GOLGI |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| homotopic and heterotypic fusion |
|
Definition
| membranes fusions from same or different compartment |
|
|
Term
|
Definition
| transmembrane proteins that help package ER to Golgi COP2 vesicles |
|
|
Term
|
Definition
| excessive bleeding from less Factors 5 and 7 |
|
|
Term
| Golgi processing of N-linked glycosylation |
|
Definition
| less mannosen more everything else |
|
|
Term
|
Definition
| happens in golgi after golgi mannosidase 2 |
|
|
Term
|
Definition
| die by age 7, cannot digest many carbs lipids and proteins as lysosomal stoarge |
|
|
Term
| How do you get to use a lyosome? |
|
Definition
autophagy
endosomes by endocytosis
phagocytosis |
|
|
Term
| 2 endocytosis membrane molecules |
|
Definition
clathrin coated pits (proteins)
caveolae (lipid rafts) |
|
|
Term
| How does LDL enter the cell? |
|
Definition
| receptor mediated endocytosis as aPROTEIn recognizes it is must be Clathrin |
|
|
Term
| how do we aquire passive immunity? |
|
Definition
| transocytosis of antibodies in small intestine, there is an antibody receptor there (FC receptor) |
|
|
Term
| What are the two early endosomes? |
|
Definition
apical and basolateral (two different sides of cell)
there is a common late one that they both fuse into |
|
|
Term
|
Definition
| they go to through own compartment like an endosome (called caveosome) and do not get exposed to low pH! |
|
|
Term
constitutive secretory pathway
regulated secretory pathway |
|
Definition
consitutire will always send its contets out
regulated need to get the signal to do this (like mast cells and histamine) |
|
|
Term
| What is the relationship between Km and affinity? |
|
Definition
|
|
Term
| What changes / stays the same in competitive vs noncompetitive inhibition? |
|
Definition
competitive - Km goes up
noncompetitive - Vmax goes down |
|
|
Term
|
Definition
B1
1 year old pediatricians are the brats
pyruvate dehydrogrenase
alpha keto gluterate dehydrogenase
thio HMP Shunt
Branch Chain |
|
|
Term
|
Definition
| a non protein compound that participates in catalysis |
|
|
Term
Lyases
Ligases
Isomerases
hydrolases
transferases
oxidoreductase |
|
Definition
break bonds (why brake? imagine bond screaming WHY!))
make bonds
rearrange structure
break bonds using water
transfer a group (kinase)
dehydrogenase, oxygenases |
|
|
Term
|
Definition
| competitive inhibitor of succinate dehydrogenase |
|
|
Term
|
Definition
|
|
Term
|
Definition
| enzymes with catalytic activity |
|
|
Term
|
Definition
problems with type 1 collagen, causing weak bones
every molecule of collagen uses 2alpha1s and only 1 alpha2
idea is that a null allele just makes less gene product and an expressed bad allele wreaks havack |
|
|
Term
|
Definition
autosomal recessive,
deteriorate quickly dead in 2-4 years,
deficiency in hexosaminaidase A |
|
|
Term
| 21-Hydroxylase deficiency |
|
Definition
| ambiguous genitalia, enzyme inactivity that makes way too much testosteroe |
|
|
Term
|
Definition
| another gene product can suffice for a deficient one |
|
|
Term
|
Definition
cat- destroys! (oxidation)
ana- builds! (reduction) |
|
|
Term
| What are the two alternate enzymes that will act on glucose as it enters the cell? |
|
Definition
hexokinase (body, high affinity)
glucokinase (liver, low affinity) |
|
|
Term
| Rate limiting step of glycolysis? |
|
Definition
phosphofructokinase-1
(phosphorylates fructose 6-P into Fructose 1,6 Bisphosphate!) |
|
|
Term
| Order of molecules in glycolysis |
|
Definition
glucose
glucose 6 P
fructose 6 P
fructose 1,6 P
two molecules: Dihydroxyacetone phosphate & Glyceraldehyde 3P
1,3-bis-Phosphoglycerate
3Phosphoglycerate
2 Phosphoglycerate
Phosphoenol pyruvate
Pyruvate |
|
|
Term
| Which glycolysis enzymes use ATP? |
|
Definition
Hexokinase or glucokinase in liver
Phosphofructokinase |
|
|
Term
|
Definition
Glceraldehyde 3-P dehydrogenase
(also adds an inorganic phosphate) |
|
|
Term
|
Definition
phosphopglycerate kinase
pyruvate kinase |
|
|
Term
|
Definition
boost: Fructose 2,6 BP (make by insulin) or AMP
down: ATP, Citrate |
|
|
Term
|
Definition
| Replaces phosphorus but dose not allow for ATP snthesis |
|
|
Term
| What does floride do to glycolysis? |
|
Definition
stops enolase reaction
2-phosphoglycerate to phospoenolpyruvate |
|
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Term
| Pyruvate kinase deficiency |
|
Definition
| 1 per 10,000 recessive inherit, hemolytic anemia as the affect isozyme is only in RBC's |
|
|
Term
| How is pyruvate kinase regulated? |
|
Definition
positive - fructose 1,6 bisP (feed forward)
negative - alanine and
cAMP (for fasting only in liver so that the liver wil lmake glucose instead of pull it from blood) |
|
|
Term
Reversible reactions to use pyruvate?
Irreversible? |
|
Definition
Reversible: Alanine or Lactate
Pyruvate to OAA to Gluconeogenesis
Pyruvate to acteyl coA |
|
|
Term
| order of enzymes, glycolysis |
|
Definition
|
|
Term
| tissue that use primary anerobic respiration |
|
Definition
| RBC, WBC, eye, kidney medulla |
|
|
Term
| What enzymes does fermentation? |
|
Definition
|
|
Term
| What happens to the lactate after it is created? |
|
Definition
| turned into pyruvate in most tissues, turned in glucose by the liver (Cori cycle) |
|
|
Term
|
Definition
| pyruvate dehydrogenase complex, active without phosphate (remember, it already has the P of PDC) |
|
|
Term
|
Definition
| activated by Ca2+ (takes off phosphate from PDC and therein activates it) |
|
|
Term
|
Definition
|
|
Term
What makes PDC active?
What hurts the PDC? |
|
Definition
active - Ca+, ADP, Pyruvate
inactive - Acetyl CoA, NADH (TCA not working if NADH is around) |
|
|
Term
|
Definition
Lactic acidosis is the main symptom
known as Leigh syndome |
|
|
Term
|
Definition
creates ribose sugars from glucose to make NADPH for synthesis pathways,
if messed up, we have bad RBC's |
|
|
Term
| What is be see a buildup of Na+ |
|
Definition
| no energy to fuel the NaK ATPase! |
|
|
Term
Glycerol phosphate shuttle
malate aspartate shuttle |
|
Definition
Glycerol - NADH to FADH2 (obviously gives less atp as it is not as strong)
Glycerol is from FATS remember the F this way
malate - NADH to NADH
carry electrons from glycolysis to to ETC |
|
|
Term
How many ATP from NADH?
How many from FADH2? |
|
Definition
|
|
Term
nmeumonic for Krebs
which ones are dehydrogenases? |
|
Definition
Citrate Is Krebs Starting Substrate For Making Oxaloacetate
3-4-6-8 IKSM |
|
|
Term
| thiamin (vitamin B1) - TPP |
|
Definition
| needed for alpha-ketoglutarateand PDC |
|
|
Term
|
Definition
| needed for acetyl CoA and succinyl coA |
|
|
Term
|
Definition
| regulated negatively by citrate |
|
|
Term
| What is the rate limiting step of the Citric Acid cycle? |
|
Definition
|
|
Term
| isocitrate dehydrogenase regulation |
|
Definition
|
|
Term
| Why are all the dehydrogenases in the TCA regulated by NADH but not Succinate dehydrogenase? |
|
Definition
| it is in the ETC and immediately used up! |
|
|
Term
|
Definition
| those that replenish pieces of the TCA cycle |
|
|
Term
| What enzyme takes pyruvate to make oxaloacetate? |
|
Definition
|
|
Term
| What happens if there is a lot of acetyl CoA around? |
|
Definition
| stop PDC, make pyruvate go to lactic acid or OAA |
|
|
Term
| What cofactors are needed for the ETC? |
|
Definition
|
|
Term
|
Definition
|
|
Term
| How does ADP and ATP get in and out of the matric? |
|
Definition
| cotransporter exchange involving the allowance of 1 H+ to enter matrix |
|
|
Term
| What is the rate limiting step of the ETC? |
|
Definition
| rate of ADP coming into the matrix! |
|
|
Term
| Name two proton uncouplers |
|
Definition
|
|
Term
| What are some inhibitors of the ETC? |
|
Definition
oligomycin, CO, CN-, Antimycin A,
Amobarbital Rotenone |
|
|
Term
| What is the common structure of antioxidants? |
|
Definition
| conjugated double bonds, often in aromatic ring |
|
|
Term
| Does mitochondira DNA have introns? |
|
Definition
|
|
Term
|
Definition
homoplasmic, ND1 subunit mutation,
blindness @ 30's, more men have problems, smoking causes onset |
|
|
Term
|
Definition
| heteroplasmic, point mutation, bad msucles, heaaches, vomiting, strokes, NADH dehydrogenase, some tRNAs, diabets, deafness |
|
|
Term
|
Definition
| heteroplasmic - point mutation in tRNAlys, epilespy in ragged-red muscle fibers, ataxia,dementia |
|
|
Term
|
Definition
| kearns-Sayre, large deletion, heart problems, ataxia, diabetes, somatic mutations |
|
|
Term
|
Definition
chronic progressive external opthalmoplegia
the MELAS tRNA mutation and large deletions, rapid loss of eye musles heteroplasmic
inheritance of point mutations
somatic deletions |
|
|
Term
mitochondrial mutations
missense
single base in tRNA
dup and dels |
|
Definition
missense - LHON
single base in tRNA-MERF, MELAS
Dup and Del - KSS, Peasrson, CPEO |
|
|
Term
| Gluconeogenesis irreversible enzymes |
|
Definition
Pathway Produces Fresh Glucose
1) Pyruvate carboxylase does not allow it to be made into acteyl CoA
2) PEP carboxykinase goes reverse to pyruate carboxylase
3) Fructose 1,6 Bisphsphatase (beats pfk-1)
4) Glucose 6 phosphatase (beats glucokinase) |
|
|
Term
|
Definition
| mild, glasctose can just leave the cells and does not have to accumulate as it is never phosphorylated, bad eyes as they have ALDOSE REDUCTASE which is osmotically active |
|
|
Term
|
Definition
| galactosemia - now is accumulates everywhere, rush of water into cell |
|
|
Term
|
Definition
|
|
Term
|
Definition
| caused by aldolase b problem after fructose has already been brought into the cell and phosphorylated. Biggest roblem is hypoglycemia as it has grabbed all the phosphates so we cannot do gluconeogenesis |
|
|
Term
| sorbitol dehydrogenase problems |
|
Definition
| glucose to sorbitol to fructose after too much glucose is in blood, good way to trap it, but now i is stuck as sorbitol which causes damage |
|
|
Term
| What does glycogen degrade to? |
|
Definition
| Glucose 1-phosphate, converted to Glucose-6 phosphate |
|
|
Term
| Glycogen storage diseases |
|
Definition
Very Poor carbohydrate metabolism
Von Gierkes
Pompes
Coris
McArdles |
|
|
Term
| Two Ez glycogen metabolism deficiencys |
|
Definition
muscle - McArdles
liver(hepatocytes) - her's disease, only see when working out
both cause hypoglycemia either when working out or between meals |
|
|
Term
|
Definition
Anderson has branching enzyme deficiency
Cori has debranching enzyme deficinecy |
|
|
Term
|
Definition
| trashes the pump (heart), lysomes cannot break down glycogen |
|
|
Term
|
Definition
| glucose 6 phosphatase - allows glucose 6 phosphate to go to glucose and leave the liver cells, serve hypoglycemia |
|
|
Term
| What three substances can undergo gluconeogenesis? |
|
Definition
lactate, alanine, glycerol
lactate and alanine can be derived from pyruvate
glycerol can be from fats (enters cycles goes to DHAP - Glyceraldehyde) |
|
|
Term
| Main control of Glycolysis vs Gluconeogenesis |
|
Definition
control PFK-1; Fructose 1,6 bisphosphatase
lots of fructose 2,6 bisphosphate will push towards PFK and glycolysis |
|
|
Term
|
Definition
incorrect mannose, lysosome proteins secreted out of cell
PKU - phen
XALT - long fatty acids can not be broken down by proteosomes
Tay Sachs - hexosamidiase in lysosome, GM2 builds up and destroys brains |
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