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Vitamins
NA
33
Biochemistry
Graduate
10/18/2012

Additional Biochemistry Flashcards


 

 


 

Cards

Term
Thiamine (2)
Definition
Vitamin B1
1. Coenzyme for Pyruvate Dehydrogenase E1
2. Coenzyme for Alpha-Keto-Glutarate Dehydrogenase (E1)
3. Coenzyme for Transketolase
Term
Riboflavin
Definition
Vitamin B2 and used in FADH2
1. Coenzyme for Pyruvate Dehydrogenase (E3)
2. Coenzyme for Alpha-Keto-Glutarate Dehydrogenase (E3)
3. Coenzyme in Complex II (SDH)
As FMN
1. Coenzyme for Complex I
Term
Pantothenic Acid
Definition
Vitamin B5 and CoA
1. Coenzyme for Pyruvate Dehydrogenase (E2)
2. Coenzyme for Alpha-Keto-Glutarate Dehydrogenase (E2)
Term
Pyridoxal Phosphate
Definition
Vitamin B6
1. Coenzyme for glycogen phosphorylase
Term
Biotin
Definition
Vitamin B7
1. Coenzyme for Pyruvate Carboxylase
Term
Niacin
Definition
Vitamin B3 and Used in NADH and NADPH
1. Coenzyme for Pyruvate Dehydrogenase (E3)
2. Coenzyme for Alpha-Keto-Glutarate Dehydrogenase (E3)
Term
Lipoic Acid
Definition
1. Coenzyme for Pyruvate Dehydrogenase (E2)
2. Coenzyme for Alpha-Keto-Glutarate Dehydrogenase (E2)
Term
Thiamine Deficiencies (2)
Definition
Wet BeriBeri
Wernicke-Korskakoff syndrome
Term
Wet Beriberi
Definition
Cardiomyopathy due to thiamine deficiency
Term
Wernicke-Korskakoff Syndrome
Definition
Alcohol inhibits uptake of thiamine through intestinal epithelium. Common in alcoholics.
Wernicke = Mental derangements
Korsakoff = Psychosis
Term
Impaired PDH (2)
Definition
Lactic Acidosis
Neurological Damage
Term
PDH Deficiency
Definition
Rare
X-linked or X-inactivation
Term
PDH Elimination
Definition
Death
Term
PDH and Fumarase deficiencies (2)
Definition
Rare
Severe neurological impairment
Death
Term
MERRF Myoclonic Epilepsy with Ragged Red Fibers (2) (4)
Definition
1. Mitochondrial Disease of Mito Inheritance
2. Point mutation in tRNA for lysine

1. Abnormal eye movements
2. Loss of hearing
3. Ragged red fibers
4. Lactic Acidosis
Term
MELAS (Mitochondrial Encephalopathy with Lactic Acidosis and Stroke (2) (3)
Definition
1. Mitochondrial disease from mito inheritance
2. Point mutation in tRNA for leucine

1. Abnormal development
2. Lactic acidosis
3. Cardiomyopathy
Term
LHON (Leber's Hereditary Optic Neuropathy) (2) (2)
Definition
1. Mitochondrial disease from mito inheritance
2. Antibodies leading to defective
Complex I proteins

1. Blindness
2. Tremor
Term
Primary Biliary Cirrhosis
Definition
1. Autoimmune disease of mitochondria
2. Antibodies to PDH complex
Term
Dilated cardiomyopathy
Definition
1. Autoimmune mitochondrial disease
2. Antibodies to Adenine Nucleotide Transporter
Term
GM Type IA: Von Gierke's Disease (1) (7)
Definition
1. Glucose-6-Phosphatase Deficiency

1. Normal Glycogen
2. Increased glycogen stores
3. Fasting hypoglycemia
4. Liver: Fatty and megaly
5. Renal: Disease and megaly
6. High lactic acid, lipids, uric acid in blood
Term
GM Type Ib (1) (8(
Definition
1. Glucose-6-Phosphate Translocase Deficiency

1. Neutropenia
2. Recurrent infections
3. Normal Glycogen
4. Increased glycogen stores
5. Fasting hypoglycemia
6. Liver: Fatty and megaly
7. Renal: Disease and megaly
8. High lactic acid, lipids, uric acid in blood
Term
Type II: Pompe (1) (3)
Definition
1. Lysosomal Alpha (alpha-->4) Glucosidase Deficiency

1. Excessive increase in normal structured glycogen in abnormal lysosomal vacuoles

1. All organs affected
2. Normal blood sugar
3. Massive cardiomegaly
3.
Term
Type III: Cori Disease (2) (3)
Definition
1. 4:4 Transferase Deficiency
2. 1:6 Glucosidase deficiency

1. Increased amount of glycogen with very short branches.
2. Fasting hypoglycemia
3. Muscle and Liver
Term
Type IV: Andersen's Disease (1) (3)
Definition
1. Branching enzyme

1. Normal amount of glycogen with very long outer branches
2. Affect liver and spleen
3. Cirrhosis and liver Failure before age 2
Term
Type V: McArdle's Disease (1)(7)
Definition
1. Skeletal muscle glycogen phosphorylase deficiency

1. Moderate increase in normal structured glycogen
2. Muscle affected only
3. Huge ADP increase after exercise
4. Weakness and cramping of muscle after exercise
5. No increased lactate
6. Myoglobinemia and Myogloburia
7. Benign but chronic
Term
Type VI: Her's Disease (1)(3)
Definition
1. Liver Glycogen Phosphorylase Deficiency

1. Increased amount of glycogen
2. Liver affected
3. Mild fasting hypoglycemia
Term
Type VII (1) (3)
Definition
Phosphofructokinase deficiency

1. Increased amount of glycogen
2. Muscle
3. Benign but cramps
Term
Type VIII (1) (3)
Definition
Phosphorylase Kinase defiency

1. Liver
2. Increased glycogen
3. Mild hypoglycemia and liver enlargment
Term
G6PDH Deficiency (3)
Definition
1. 10% US Black Males are carriers
2. Malarial belt adaptation that causes infected RBC's to lyse quicker in presence of ROS's.
3. Increased oxidative stress through sulfa drugs, antimalarials, and fava beans increase hemolysis.
Term
Essential Fructosuria (2)
Definition
1. Lack of fructokinase
2. Autosomal recessive

1. Benign
2. Fructose (reduced sugar) in urine
Term
hereditary Fructose Intolerance (2) (4)
Definition
1. Absence of Aldolase B
2. Autosomal recessive

1. Jaundice
2. Hypoglycemia
3. Vomiting, Liver and kidney problems
4. Lacticacidemia
Term
Non-classic galactosemia (2) (3)
Definition
1. Galactokinase deficiency
2. Autosomal recessive

1. Galacitol accumulation causing cataracts
2. Galactose in urine and blood
3. Fairly benign
Term
Classic Galactosemia
Definition
1. GALT deficiency
2. Autosomal recessive

1. G-1-P and Galacitol accumulate in nerve, lens, liver, and kidney
2. Cataracts
3. Liver damage
4. Mental retardation
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