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FA Biochem

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Inhibit electron transport
rotenone, cyanide, antimycin A, CO
Replenishes TCA or can be used in gluconeogenesis
oxaloacetate
Recognize and remove bases in excision repair
glycosylases
Adds O-oligosaccharides to serine and threonine
golgi
Cofactor for proprionyl coA -> methylmalonyl coA
biotin
B vitamin deficiency where you see abnormal myelin
B12
Cofactor for acetyl coA -> malonyl coA
biotin
Dermatitis, enteritis, alopecia, adrenal insufficiency
B5 deficiency
Diarrhea, dermatitis, dementia
pellagra
What is a collagen fibril
crosslinked tropocollagen
Carries aldehydes
TPP
Makes tRNA
RNA pol III
Uses NADPH to reduce glutathione
glutathione reductase
Backup of these two things results in lactic acidosis (from pyruvate dhase deficiency)
pyruvate and alanine
Scrutinizes amino acid after it is bound to tRNA. Defect leads to insertion of wrong amino acid.
Aminoacyl-tRNA synthetase
Cystic fibrosis, sprue, mineral oil intake
fat soluble vitamin deficiencies
Malignant carcinoid can lead to a deficiency of what vitamin
B3
Assembles proteoglycans
golgi
These tissues participate in gluconeogenesis
liver, kidney, intestinal epithelium
Used for energy to "charge" tRNA
ATP
Direction of DNA pol III exonuclease activity
3-5
What is the function of lysyl oxidase
crosslink tropocollagen at lysine-hydroxylysine to make collagen fibrils
The folic acid precursor in bacteria
PABA
What drug can cause B3 deficiency
INH
Deficient in fructose intolerance
aldolase B
Cofactor for homocysteine methylation
B12
How much ATP is used by HMP shunt
none
Major component of bile
phosphatidylcholine
Cofactor for transketolase in HMP shunt
B1
Uterus, fetal tissue, granulation tissue
type III collagen
Sulfates sugars and tyrosines on proteins
golgi
Used for catabolic processes to carry reducing equivalents away
NAD+
Worst type of Ehlers-Danlos
type III
Needed for regeneration of SAM
B12
Cofactor for transamination, decarboxylation, heme synthesis
B6
Inhibit mitochondrial ATPase, increase the proton gradient
oligomycin
Where does fructose-1,6-bisphosphatase work
cytosol
Cofactor needed for breakdown of odd-chain FA
biotin
Important for synth of nitrogenous bases
folic acid
Help assemble ribosomal subunits
initiation factors
Cofactor for oxidative decarboxylation of alpha keto acids
B1
Converts H2O2 to HOCl
myeloperoxidase
Where does vitamin C-dependent hydroxylation of proline and lysine take place
ER
Trafficking protein for trans-golgi -> lysosome, and plasma membrane -> endosomes
clathrin
Major component of myelin
phosphatidylcholine
Ergocalciferol
D2 (from milk)
Converts O2 to O2 radical
NADPH oxidase
B vitamin that is a cofactor for acyl transfers
B5
Activates pyruvate dehydrogenase complex (3)
increased NAD+, increased ADP, increased calcium
Which end bears the triphosphate, 3 or 5
5
Converts PEP to pyruvate
pyruvate kinase
Trade a purine for a purine
transition
Malate shuttle
38 ATP
Opens DNA at promoter site
RNA pol II
Cheilosis, corneal vascularization
B2 deficiency
Where is insoluble tropocollagen formed
outside fibroblasts
What is a triple helix of three collagen alpha chains
procollagen
Convulsions, irritable, peripheral neuropathy
B6 deficiency
High affinity, low capacity, feedback inhibition
hexokinase
Trafficking protein for anterograde RER -> cis-golgi
COP II
Converts oxaloacetate to PEP in the cytosol
PEP carboxykinase
Site of RNA processing
nucleus
Converts NADP+ to NADPH
G6PD
Deficiency of this vitamin may predispose to alcoholic cirrhosis
zinc
Divide rapidly with a short G1
labile cells
How is G6PD deficiency inherited
autosomal recessive
Deficient in essential fructosuria
fructokinase
Result of diverting pyruvate to lactate, and OAA to malate in the liver
inhibition of gluconeogenesis -> alcoholic hypoglycemia
Converts H2O2 to H2O
catalase/glutathione peroxidase
What cofactor is needed for gluconeogenesis
biotin
Cofactor for pyruvate -> oxaloacetate
biotin
Energy for RBCs, leukocytes, kidney medulla, lens, testes, cornea
anaerobic glycolysis
Low affinity, high capacity, no feedback inhibition
glucokinase
Modifies N-oligosaccharides on asparigine
golgi
Converts O2 radical to H2O2
superoxide dismutase
Converts pyruvate to acetyl coA
pyruvate dehydrogenase
Vimentin stain
connective tissue
Treatment of pyruvate dehydrogenase deficiency (possibly alcoholics with B1 deficiency)
increased fat intake, lysine, leucine
Optic neuropathy, subacute combined degeneration, paresthesias, glossitis
B12 deficiency
Decrease permeability of membrane, decrease proton gradient, decrease O2 consumption
uncoupling agents
Where does glucose-6-phosphatase work
ER
Makes mRNA
RNA pol II
Combines with snRNPs to form the spliceosome
primary transcript
Causes fatty liver in alcoholics
altered NADH/NAD ratio, shunting away from glycolysis and toward FA synthesis
Transfers lactate to the liver from RBCs
cori cycle
Hemoglobin precipitated in RBCs
Heinz bodies
How many amino acids does the ribosome move to make an amino acid move from A to P site
3
Protein needed for receptor mediated endocytosis
clathrin
Major component of RBC membranes
phosphatidylcholine
Loss of appetite and stupor due to a vitamin OD
vitamin D
Needed to make ribose-5-P, G3P, F6P
transketolases (require B1)
Shortest phase of the cell cycle
mitosis
Antioxidant vitamin
vitamin E
Stimulates pyruvate kinase
F16BP
Site of addition of man-6-p to lysosomal enzymes
golgi
UCP, 24-DNP, aspirin
uncoupling agents
Alpha keto acids
pyruvate, a-ketoglutarate
Carries 1-carbon units
THF
Carries amino groups from liver to muscle
alanine
How many ATP per acetyl coA that enters the TCA cycle
12
ATP + methionine
SAM
Catalyzes gamma-carboxylation of glutamic acid
vitamin K
B vitamin needed for TCA cycle
B1
Makes rRNA
RNA pol I
Synthesize enzymes and peptide neurotransmitters
RER nissl bodies
Used in anabolic processes, respiratory burst, P-450
NADPH
What vitamin is needed to derive B3 from tryptophan
B6
Complexes with a fibrillin scaffolding
tropoelastin
Trafficking protein for retrograde golgi -> ER
COP I
Deficient in von gierke's
glucose-6-phosphatase
Deficient in mild galactosemia
galactokinase
Deficient in severe galactosemia
gal-1-P uridyltransferase
INH and OCP interfere with
B6
Energy used for translocation of the ribosome
GTP
Direction of DNA pol I exonuclease activity
5-3
Facilitates iron absorption by keeping it in the reduced state
biotin
Site of O-linkage
serine and threonine residues
Desmin stain
muscle
Two amino acids abundant in elastin
nonhydroxylated proline and lysine
Used for anabolic processes to supply reducing equivalents
NADPH
Used in the esterification of cholesterol
phosphatidylcholine (LCAT)
Cofactor for one carbon transfer in methylations
folic acid
Storage of B12
liver
Which end of tRNA does amino acid attach to
3
Polyneuritis and muscle wasting
dry beriberi
Limiting reagent in ethanol metabolism
NAD
Needed as a cofactor for dopamine -> NE
vitamin C
Inhibits pyruvate kinase
alanine, ATP
B vitamin that is a component of fatty acid synthase
B5
Neurofilament stain
neurons
G3P shuttle
36 ATP
Is there proofreading for RNA
no
Trade a purine for a pyrimidine
transversion
Inhibits pyruvate dehydrogenase
acetyl coA, ATP, NADH
Inhibits RNA polymerase II
a-amanitin
Microtubule polymerization defect leading to problems with phagocytosis
chediak-higashi syndrome
GFAP
neuroglia
Name of primary transcript before the cap and the tail
hnRNA
Where is procollagen formed
golgi, by glycosylation
Inhibits PFK-1
citrate, ATP
Findings in pyruvate dehydrogenase deficiency
neurologic defects
What codon does amino acid attach to on tRNA
CCA
Converts pyruvate to oxaloacetate in mitochondria
pyruvate carboxylase

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