Cloned from: USMLE Biochem

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TCA cycle intermediates
\\\"Can I Keep Selling Sex For Money
Regulated glycolytic enzymes
\\\"Hexokinase (-G6P) Glucokinase (+insulin) PFK1 (-citrate -ATP +AMP +F-2
Regulated TCA cycle steps
\\\"Citrate synthetase (-ATP) Isocitrate dehydrogenase (+ADP
Lesch-Nyhan syndrome
\\\"HGPRT deficiency
I cell disease
\\\"Lack of mannose-6-phosphate transfer enzyme in golgi network means can\\\'t tag lysosomal enzymes for traffic to lysosome. Get secreted instead->coarse facies
Energy from TCA cycle per acetyl CoA
3 NADH --> 9 ATP 1 FADH2 --> 2 ATP 1 GTP --> 1 ATP 12 ATP/cycle via oxidative phosphorylation
\\\"Mild: Galactokinase deficiency->galactitol->childhood cataracts Severe: Gal-1P uridyl transferase deficiency-> very high galactitol->liver damage
\\\"Fructokinase deficiency: benign fructosuria Fructose intolerance: Lack of aldolase B to convert F1P to DHAP and glyceraldehyde->vomiting w/fructose load
Cofactors for PDH and a-KGDH
Vitamin B1->thiamine->TPP Vitamin B2->riboflavin->FAD+ Vitamin B3->niacin->NAD+ Vitamin B5->pentothenate->CoA Lipoic acid
Cori cycle
1) Anaerobic glycolysis in muscle to pyruvate 2)Pyruvate -> lactate to regenerate NAD+ 3) Lactate to liver via blood 4) Lacate converted back to pyruvate and then to glucose by gluconeogenesis (6 ATP) 5) Glucose sent back to muscle in blood
Energy yield of anaerobic glycolysis
2 ATP Reducing equivalents in NADH used to convert pyruvate to lactate via LDH to regenerate NAD+ to keep running glycolysis
Hexokinase vs glucokinase
\\\"Hexokinase: all cells
S-adenosyl methionine ATP+Met-->SAM->->Homocysteine Need B12 & folate to regenerate methionine from ATP Regeneration of methionine is how B12 converts dietary folate into form usable by purine synth and thymidylate synthase
GPCRs that signal via Gs
Gs stims cAMP synth B1->inotrope/chronotrope B2->SMC relaxation H2->stomach V2->aquaporin insertion in kidney D1
GPCRs that signal via Gq
Gq > PLC > DAG+IP3 > PKC & Ca2+ H1->allergy a1->vasoconstrict V1->vasoconstrict M1 M3
GPCRs that signal via Gi
Gi inhibs cAMP synth M2 a2 D2
Collagen types
\\\"Type 1: classic (bone
Electron transport chain
NADH > e- > Complex I > H+ CoQ FADH2 > e- > Complex II >CoQ complex III > H+ Cyt C Complex IV > H+ + O2 H+ > ATP synthase > ATP
Inhibs ATP synthase > can\\\'t dissipate H+ gradient > ETC machinery gets backed up and stopped > ROS
Inhibs e- transport > stops ETC > reduces proton gradient
Inhibs e- transport > stops ETC > reduces proton gradient
Irreversible gluconeogenesis enzymes
\\\"Pyruvate carboxylase (mitochondria PEP carboxykinase Fructose-2
von Gierke\\\'s disease
Type I glycogen storage disease: Glucose-6-phosphatase deficiency Liver can\\\'t export glucose: -Glycogen accum->hepatomegaly -Hypoglycemia -Lactic acidosis
Essential amino acids
PVT TIM HALL Phenylalanine Valine Tryptophan Threonine Isoleucine Methionine Histidine Arginine Leucine Lysine
Phenylalanine Valine Tryptophan Threonine Isoleucine Methionine Histidine Arginine Lysine Leucine
Purely ketogenic amino acids
Leucine & lysine only. Both are also essential
Purely glucogenic essential AA:
\\\"V-MATH Valine
Urea cycle
Black urine
\\\"Alkaptonuria: can\\\'t break down homogentisic acid
\\\"Musty odor
pale skin
Cystinuria frequency
Cystinuria defect
\\\"COLA Defect of AA transporter responsible for resorption of Cysteine
Components of sucrose
Fructose + glucose
Components of lactose
GaLactose + glucose
Function of ApoA1
Cofactor for LCAT
Function of ApoB
Binds LDLR
Function of ApoCII
Cofactor for lipoprotein lipase
Function of ApoE
Cofactor for lipoprotein binding to receptor for uptake
Cherry red spot
\\\"Tay-Sachs disease
Rate limiting enzyme of De Novo pyrimidine Synthesis
Aspartate transcarbamylase (AT Case)
Rate limiting enzyme of De Novo Purine Synthesis
Glutamine PRPP amidotransferase
Rate limiting enzyme of Glycolysis
Phosphofructokinase-1 (PFK-1)
Rate limiting enzyme of Gluconeogenesis
pyruvate carboxylase
Rate limiting enzyme of TCA Cycle
Isocitrate dehydrogenase
Rate limiting enzyme of Glycogen synthesis
glycogen synthase
Rate limiting enzyme of Glycogenolysis
Glycogen phosphorylase
Rate limiting enzyme of HMP Shunt
Glucose -6-phosphate dehydrogenase (G6PD)
Rate limiting enzyme of Fatty Acid synthesis
Acetyl-CoA Carboxylase (ACC)
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