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43 Cards in this Set
- Front
- Back
- 3rd side (hint)
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Specific residues that are cross-linked by lysyl oxidase?
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Lysine and hydroxylysine
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Universal electron acceptors used for catabolic reactions?
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NAD+
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Universal electron acceptors used for anabolic reactions?
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NADPH
Used in steroid and fatty acid synthesis From G6PD |
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NADPH used in what reactions?
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p450
Respiratory burst Anabolic processes (steroid and fatty acid synthesis) Glutathoine reductase Reduction reactions |
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Arsenic
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Inhibits lipoic acid
Clinical Findings: Vomiting, garlic breath, rice water stool |
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Pyruvate dehyrogenase deficiency
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Clinical findings:
Lactic acidosis Neurological defects Increase in pyruvate and alanine Treatment: Diet of ketogenic amino acids (lysine and leucine) |
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Oxidative phosphorylation poisons that block electron transport
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Rotenone
CN CO antimycin A |
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Oxidative phosphorylation poisons that inhibit ATPase
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Oligomycin
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Where does gluconeogenesis occur?
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Primarily in the liver
But Also where? |
Kidney and intestinal epithelium
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Enzymes important for gluconeogenesis
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Pyruvate carboxylase
PEP carboxykinase Fuctose-1,6-bisphosphatase Glucose-6-phosphatase |
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Sorbitol dehydrogenase
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Converts sorbitol to fructose
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Glucogenic AA
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Met
Val Arg His |
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Glucogenic/Ketogenic AA
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Ile
Phe Thr Trp (PITT) |
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Basic AA
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Arg
Lys His Arg is most basic His has no charge at body pH |
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AA important during periods of growth
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Arg
His |
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Essential Amino Acids
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Phe
Val Thr Trp Ile Met His Arg Leu Lys |
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Symptoms of hyperammoniaemia
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Slurring of speech
Somnolence Cerebral edema Tremor Vomiting Blurring of vision |
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Cofactor for conversion of histidine to histamine
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B6
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Derivatives of Arginine
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Urea
NO Creatinine |
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Derivatives of Glutamate
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GABA (requires B6) via glutamate decarboxylase
Glutathione |
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Deficiencies in PKU
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Phenylalanine hydroxylase or tetrahydrobiopterin
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Why do PKU children have fair skin?
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Excess phenylalanine inhibits tyrosinase, which is the enzyme responsible for the synthesis of melanin
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Homocystinuria
Mechanism & Clinical Findings |
3 forms
1) Decrease affinity of cystathione synthase for pyridoxal phosphate (inc B6 in diet) 2) Homocysteine methyltransferase deficiency 3) Cystathione synthase deficiency (dec Met, inc Cys, inc B12) Clinical findings: MR, tall stature, down and in subluxation of lens, atherosclerosis (risk MI and stroke), kyphosis, osteoporosis, increased homocysteine in urine |
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Zellweger Syndrome
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Peroxisomal disease
Infants cannot form myelin in CNS Clinical findings: Hypotonia, hepatosplenomegaly, seizures, MR, early death |
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Refsum's Disease
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Defect in peroxisomal alpha-oxidation
Neuro disturbances due to buildup of phytanic acid Treat: Strict avoidance of chlorophyll in diet |
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DNA polymerase I
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Prokaryotic
Degrades RNA primer Has a 5' --> 3' exonuclease |
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DNA polymerase III
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Prokaryotic only
5'--> 3' elongation 3'--> 5' exonuclease |
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Transketolase
(pathway, location, cofactors) |
Enzyme in the HMP shunt
Requires TPP cofactor Found in Cytoplasm |
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Function of ApoA-1
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Activates LCAT
LCAT catalyzes esterfication of cholesterol |
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Function of ApoB100
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Binds LDL receptor and mediates VLDL secretion
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Function of ApoC-II
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Cofactor for lipoprotein Lipase
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Function of ApoE
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Facilitates uptake of Extras (remnants)
VLDL and chylomicron |
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Function of ApoB-48
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Mediates chylomicron secretion
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Tuberous Sclerosis
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Shagreen Patches
Ash Leaf Macules Cardiac Rhabdomyomas Renal Cysts Renal Angiomyolipomas Seizures MR Adenoma Sebaceum Cortical and retinal hamartomas Subepindymal hamartomas Astrocytomas Mitral Regurg SALSA HEART Shagreen patches Ash Leaf macules Sebaceous Ademoa Hamartoma of CNS, skin, organs Angiomylipoma Renal Cysts/Rhabdomyoma of heart Tubers of CNS |
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Function of B1
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Cofactor for
1) PDH complex 2) Alpha-ketoglutarate Dehydrogenase 3) Transketolase 4) Branched-chain AA dehydrogenase |
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Rate-determining Enzyme
TCA |
Isocitrate dehydrogenase
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Rate-determining Enzyme
Glycogen synthesis |
Glycogen synthase
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Rate-determining Enzyme
De novo pyrimidine synthesis |
Carbamoyl phosphate synthetase II
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Rate-determining Enzyme
De novo Purine synthesis |
Glutamine-PRPP amidotransferase
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Rate-determining Enzyme
Urea cycle |
CPSI
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Rate-determining Enzyme
FA synthesis |
Acetyl-CoA Carboxylase
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Rate-determining Enzyme
FA oxidation |
Carnitine acyltransferase I
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Rate-determining Enzyme
Ketogenesis |
HMG CoA synthase
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