Biochemistry Memorization Flashcards
LSD: Hunter
Iduronate Sulfatase
LSD: Hurler & ScheieA
a-L-Iduronidase
LSD: I-Cell Disease
Multiple enzymes due to a defect in the M-6-P targeting system.
LSD: Pompe
Acid a-glucosidase
LSD: Mucolipidosis VII
B-Glucuronidase
Function of cofactor?
CoA/AcetylCoA
Transfers acyl groups
Function of cofactor? Where on PDH?
Thiamine Pyrophosphate
Decarboxylation and 2 carbon transfer. On E1.
Function of cofactor? Where on PDH?
Lipoic Acid/Lipoamide
Acetyl transfer. On E2.
Function of cofactor? What enzyme?
Biotin
Carboxylation. Pyruvate Carboxylase (gluconeogenesis).
Function of cofactor? What pathway(s)?
NAD+/NADH
Electron transfer for OXIDATION.
Function of cofactor?
NADPH/NADP+
Electron transfer for REDUCTION.
Coenzyme Deficiencies
Niacin (Vitamin B3)
Dermatitis
Diarrhea
Dementia
(Three-Ds)
Also called pellagra.
Coenzyme Deficiencies
Thiamine (Vitamin B1)
Ophthalmoplegia
Gait difficulties
Confusion
Beri Beri or Wernicke-Korsakoff
Coenzyme Deficiencies
Riboflavin (Vitamin B2)
Cheilosis
Glossitis
Coenzyme Deficiencies
Lipoate
Targeted by arsenic:
Pyruvate DH & a ketoglutarate dehydrogenases DH
Toxins of glycolysis–what enzyme?
2-deoxyglucose
hexokinase
Toxins of glycolysis–what enzyme?
arsenate
G3P-DH
Toxins of glycolysis–what enzyme?
Fluoride
Enolase
Glycogen Storage Disease
Type I
- Name
- Enzyme
- Location
- Glycogen changes
- Features
- Von Gierke
- Glucose-6-phosphatase
- Liver and kidney
- Amount increase, normal structure
- Severe hypoglycemia, hepatomegaly
Glycogen Storage Disease
Type II
- Name
- Enzyme
- Location
- Glycogen changes
- Features
- Pompe
- a-1,4-glucosidase
- Everywhere
- Increased amount, normal structure
- Cardiorespiratory failure
Glycogen Storage Disease
Type III
- Name
- Enzyme
- Location
- Glycogen changes
- Features
- Cori
- Debranching enzyme
- Muscle, liver
- Increased amount, short outer branches.
- Mild hypoglycemia and hepatomegaly (Like Type VI, a mild Type I)
Glycogen Storage Disease
Type IV
- Name
- Enzyme
- Location
- Glycogen changes
- Features
- Anderson
- Branching Enzyme
- Liver
- Normal amount, long outer branches
- Hepatomegaly/fatal.
Glycogen Storage Disease
Type V
- Name
- Enzyme
- Location
- Glycogen changes
- Features
- McArdle
- Phosphorylase
- Muscle
- Increased amount, normal structure
- Exercise problems, cramps (Like Type VII)
Glycogen Storage Disease
Type VI
- Name
- Enzyme
- Location
- Glycogen changes
- Features
- Hers
- Phosphorylase
- Liver
- Increased amount, normal structure
- Mild hypoglycemia and hepatomegaly (Mild Type I)
