Biochemistry Flashcards
Wernicke-Korsakoff
Thiamine / Vitamin B1 deficiency
Primary lipid of myelin
Galactocerebroside (Galc!
Fatty acid is joined to sphingosine via ______ linkage to form a ceramide.
Amide
Sphingomyelinase deficiency
Niemann-pick Disease
Major glycosphingolipid of brain and other nervous tissues
Cerebroside
Rate-limiting step in cholesterol synthesis
HMG-CoA to mevalonate
(HMG-CoA reductase)
Number of Carbons in cholesterol
27
Deficiency in Smith-Lemli-Opitz syndrome
7-dehydrocholesterol reductase
Alkylphospholipid
Platelet Activating Factor
Orlistat MOA
Reversible lipase inhibitor
Zellweger syndrome: defect in ______
Peroxisomes
Deficiency in Gaucher Disease
B-Glucosidase (Glucocerobrosidase)
Selenium deficiency cardiomyopathy
Keshan disease
Dietary deficient in Menkes syndrome
Copper
Rate limiting step of the urea cycle
CPSI (carbamoyl phosphate synthetase I)
Oxidative deamination of glutamate occurs in the __________.
Liver mitochondria
Major gluconeogenic amino acid
Alanine
Urea cycle occurs in which organ?
Liver
Hartnup disease:
impaired transport of what amino acid
Tryptophan
Enzyme associated with gyrate atrophy of choroid and retina
Ornithine transaminase
Parent porphyrin of heme
Protoporphyrin III
Rate limiting enzyme in heme production
ALA synthase
(Aminolevulinate)
Only pathway that produces CO
Heme catabolism
Rachitic rosary - rib prominence
Rickets
Dermatitis
Diarrhea
Dementia
Pellagra (Vit B3 - Niacin deficiency)
Vit B12 deficiency (cyanocobalamin)
Pernicious anemia
Disease due to body’s inability to absorb tryptophan
Hartnup Disease
Anemia due to deficiency in Folic acid (Vit B9)
Megaloblastic anemia
Frosted glass cytoplasm
Monocytes
The only non protein component of the ETC
Ubiquinone
______ binds to lipoic acid of pyruvade dehydrogenase complex making it inactive
Arsenic
Enzyme inhibited by Fluoroacetate
Aconitase (citrate —> isocitrate)
Enzyme inhibited by Arsenite and Ammonia
A-ketoglutarate dehydrogenase (a-ketoglutarate —> succinyl CoA)
Different configuration around 1 specific carbon syom (except the carbonyl atom)
Epimer (ex: glucose and mannose)
Different configuration around the carbonyl atom
Anomers
Structures that are mirror images of each other
Enantiomer aka Stereoisomers or optical isomers
Compounds that have the same chemical formula
Isomers (ex: D-glucose, D-fructose)