Goljan High Yield Biochemistry EC Flashcards
Rate limiting reaction in cholesterol synthesis
HMG Co reductase
Findings in PKU
Mousy odor
Tyrosine missing hence it must be supplied in the diet
Can diagnose by amniocentesis and finding the abnormal gene
Eliminate phenylalanine from diet (no nutrasweet)
I cell disease
Inability to phosphorylate the mannose residues of potential lysosomal enzymes in the golgi
Cannot be taken up by lysosomes to degrade complex substrates
Number of glucoses necessary to build palmitic acid (16C compound)
4 glucoses
Each glucose produces 2 acetyl CoA w/ 2 carbons each
Insulin lack in DKA
Decreased glycolysis, glycogenesis, fatty acid synthesis, storage of fat in adipose
Uncoupling agents (alcohol, salicylates)
Produces brown fat from increased heat from reactions trying to increase the generation of more protons to make ATP
Von Gierke’s
decreased glucose-6-phosphatase (gluconeogenic enzyme) with hypoglycemia and increase in glucose-6-phosphate with production of normal glycogen in the liver and kidneys
Stimulation tests with glucagon, fructose.
Cannot increase glucose levels owing to missing enzyme
Biochemical processes in both cytosol and mitochondria
Urea cycle, heme synthesis, gluconeogenesis
Female with pheochromocytoma, what dietary alteration?
Decrease phenylalanine and tyrosine in the diet
Pregnant female with PKU, what diet?
Low phenylalanine
High tyrosine
(avoid neutrasweet)
Lesch Nyhan
Absent HGPRT
Self-mutilation, hyperuricemia, mentalretardation
Glucokinase
Only in the liver
High Vm and Km
Not inhibited by G6P
Hexokinase
In all tissues
Low Vm and Km
Inhibited by G6P
Branched chain amino acids/Maple syrup urine disease
Only muscle van metabolize maple syrup urine disease
Key enzyme in gluconeogenesis
Fructose-1-6-bisphosphatase (catalyses conversion of F16BP to F6P)
Locations of glucose-6-phosphatase (gluconeogenic hormone)
Liver, kidney, intestinal epithelium
Shuttles and what they carry
Carnitine (even chained fatty acids)
Malate (NADH)
Functions of LDL
Vit. D synthesis
Steroid synthesis
Cell membranes
Synthesis of bile salts
Acetyl CoA uses
FA synthesis
CH synthesis
Ketone body synthesis
(NOT a substrate for gluconeogenesis)
Urea cycle
Method of eliminating ammonia
Located in hepatocyte
Epinephrine given and only small branches chains found
Debrancher deficiency
Origin of apolipoproteins
apo-100 =Liver
apo-48=intestine
Rate limiting step in glycogenolysis
glycogen phosphorylase
Reason why liver can not use ketones for fuel
liver cannot activate acetoacetate in the mitochondria which requires succinyl CoA: Acetoacetate CoA transferase in order to convert AcAc into acetoacetyl CoA
McArdles disease
absent muscle phosphorylase
Increased glycogen in muscle
No increase in lactic acid after exercise
Pregnant woman is a beer drinker, what supplements does she need?
Folate (alcohol increases loss)
Fetal alcohol syndrome possible
(Irone is NOT affected by beer drinking)
Insulin
Key hormone in fed state
Glucagon
Key hormone in fasting state
Mannose-6-phosphate
Involved in transfer of dolichol in the RER in the synthesis of O-linked glycosides
Major source of NADPH
HMP shunt
Malate dehydrogenase reaction to a lesser extent
Mutation changes an amino acid sequence, which one would have the greatest effect on migration in a serum protein electrophoresis?
One with the most negative charges (acidic)=Glutamine
Most basic=Arginine
Mechanism of ketoacidosis in DKA
Increased beta oxidation of fatty acids and production of acetyl CoA (used by liver to synthesize ketone bodies)
Promoter location
Linear gene drawing with labels, pick upstream locations
Energy source for protein synthesis
GTP
Isoenzyme with 2 genes, 4 subunits
LDH isoenzymes LLLL LLLH LLHH LHHH HHHH
Second messengers
ANP=cGMP
Insulin=TK
Nicotinic=ion channels
Best method of detecting relatedness of new bacteria
Restriction fragment length polymorphism
Enzyme used for PCR
DNA polymerase
Source of glucose in a brain in starvation
Alanine (transaminated to pyruvate)
Glutamine
Most abundant amino acid
Carries ammonia in nontoxic form
Tyrosine
Thyroid hormones
Melanin
Neurotransmitters
Decreased in PKU
Glycine
Inhibitory neurotransmitter
Blocked by tetanus toxin
Heme synthesis
Synthesis of bile acids
Hepatic encephalopathy
Increase in aromatic amino acids (phenylalanine, tyrosine, tryptophan [PTT])
Increased synthesis of false neurotransmitters (GABA, octopamine) in hepatic encephalopathy
Branched chain amino acids inhibit synthesis of false neurotransmitters (given to Rx)
