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Rate Limiting Ezymes

USMLE step 1 rate limiting enzymes

QuestionAnswer
RLS OF Glycolysis converts glucose C6H12O6, into pyruvate Phosphofructokinase-1 (PFK-1) In Cytosol
RLS OF Gluconeogenesis Fructose-1,6 bisphosphatase In Cytosol
RLS OF TCA cycle Isocitrate Dehydrogenase
RLS OF Glycogen Synthesis Glycogen Synthase
RLS OF Glycogenolysis Glyogen Phosphorylase Deficient in McArle's Disease
RLS OF HMP Shunt Glucose-6-phosphate dehydrogenase (G6PD)
RLS OF De Novo Pyrimidine Synthesis Pg 68 Carbamoyl Phosphate Synthase II (CPS II)
Carbamoyl Phosphate Synthase II (CPS II) is done in the? Cytosol
Carbamoyl Phosphate Synthase I is done in the? Mitochondria
RLS OF De Novo Purine Synthesis Pg 68 Glutamine- PRPP Amidotransferase
RLS OF Urea Cycle Carbamoyl Phosphate Synthase I (CPS I)
RLS OF Fatty Acid Synthesis Acetyl-CoA Carboxylase (ACC)
RLS OF Fatty Acid Oxidation Carnitine Acetyltransferase I
RLS OF Ketogenesis HMG-CoA Synthase
RLS OF Cholesterol Synthesis HMG-CoA Reductase
Carbamoyl Phosphate Synthase II (CPS II) is done in the? Cytosol
Carbamoyl Phosphate Synthase I is done in the? Mitochondria
De Novo Purine Synthesis Pg 68 Glutamine- PRPP Amidotransferase
RLS OF Urea Cycle Carbamoyl Phosphate Synthase I (CPS I)
RLS OF Fatty Acid Synthesis Acetyl-CoA Carboxylase (ACC)
RLS OF Fatty Acid Oxidation Carnitine Acetyltransferase I
Ketogenesis HMG-CoA Synthase
RLS OF Cholesterol Synthesis HMG-CoA Reductase
Pyruvate goes to Oxaloacetate by? Pyruvate carboxylase Need Biotin,ATP, and CO2 (In Mitochondria) Activated by acetyl-CoA
Oxaloacetate goes to Phosphoenolpyruvate (PEP) by? Phosphoenolpyruvate (PEP) Carboxykinase (in Cytosol) Requires GTP
Ethanol to Acetaldehyde by? Alcohol dehydrogenase (inhibited by Fomepizole) (Cytosol) Ethanol to Acetaldehyde = NAD to NADH In the Cytosol
Acetaldehyde to Acetate by? Acetaldehyde Dehydrogenase (inhibited by Disulfram/Antibuse) (mitochondria) NADH increased
Fructose to Fructose 1P by? Fructokinase Using ATP to ADP Deficiency is Essential Frucotosuria
Fructose 1p goes to Dihydroxyacetone and Glyceraldehyde by________ which goes to Glyceraldehyde 3-P Aldolase B Deficiency is Fructose intolerance
Galactose goes to Galactitol by? Aldose reductase (cataracts)
Galactose goes to Galactose 1P by? Galactokinase Using ATP to ADP Deficiency is Galactokinase deficiency
Galactose 1-P goes to Glucose 1-p By? Galactose 1 Phosphate Uridyl Transferase Using UDP-Glu to UDP-Gal (Severe galactosemia)
RLS of Heme Synthesis Aminolevulinic Acid Synthase
RLS of Bile Acid Synthesis 7-Alpha hydroxylase
Increase in Glucose 6 Phosphate von-Gierke's
Glucose 6-phosphate to Glucose which happens during Gluconeogenesis Via Glucose 6 Phosphatase (Von Gierke's) In ER
Glucose to Glucose 6 Phosphate via Hexokinase / Glucokinase In the ER
Glucose 6 Phosphate to 6-Phosphogluconolactone via Glucose 6 Phosphate dehydrogenase (G6PD)
Pyruvate goes to Acetyl-Coa via Pyruvate dehydrogenase
Phosphoenolypyruvate goes to Pyruvate in Glycolysis Via Pyruvate Kinase
Created by: Trinimd