biochem of vitamins Flashcards
Water soluble vs fat soluble
Water soluble (B1 thiamin, B2, riboflavin, B3 niacin, Pantothenic acid B5, Biotin B7, B6 pyrodoxin, Folate B9, B12, Vit C)- rapidly excreded B12 liver stores
Fat soluble: Vit A (3 retinoids), Vitamin D, Vitamin E (tocos), Vitamin K (phyllo, mena quinones), need bile, lipases and dietary fat, toxicity more common (esp starge fat), Eand K, malabsorption syndromees- Cystic fibrosis, pancreatic exocrine insufficiency, celiac disease, mineral oil intake
Vitamins involved in energy metabolism
B1, B2, Pantothenic acid, Biotin, B6 (B1, 2 3 5 6 7)
Glucose–> Glycolysis–> TCA (ATP production): B1 (TPP), B2 (FADH2), B3 (NADH), B5 (coenzyme A)
Fatty acids–> Beta oxidation: B2 (FAD), B3 (NAD), B5 (coenzyme A)
Gluconeogenesis: Biotin (B7), B3 NAD
Everything with Proteins- B6
Thiamin Vitamin B1
Active coenzyme form - Thiamin pyrophosphate TPP
Needed for PDH, BRAIN needs glucose
TCA akgdh also needs b1
GLycolysis
PDH needs B1, B2, B3, B5, lipoic acid (glycolysis and TCA)
HMP Transketolase (PPP) Also needs B1– Provides NADPH, and Ribose for fatty acid and cholesterol synthesis, maintain reduced GSH for antioxidant protection, Nicleotide synthesis
RBCs, eyes oxidant stressors
Metabolism of BCAA requires TPP B1, Maple syrup Urine disease (defect in BCaKA DH),
B 1 deficiency
Classic deficiency0 Beri Beri- (B1B1), i cant i cant, neuropathy
Dry beriberi- symmetrical impairment of sensory and motor functions of extremities esp lower extremitiry, symmpetrical muscle wasting
Wet/ edamatous beri beri- cardiomyopathy, high output CF, edema
Rice bran
Wernicke-Korsakoff syndrome
B1 deficiency Wernicke Encephalopathy (Acute life threatening)- confusion, ophalmoplegia/nystagmus, ataxia
Korsakoff syndrome (chronic neurologic condition)- confabulation, personality change, memory loss, especially anterograde amnesia
Alcoholism induced- poor diet, impaired intestinal absorption, impaired cellular utilization of thiamin
Malnourished: HIV/AIDs, cancer, hyperemesis, long term dialysis, diuretics
GIve B1 and Magnesium!!!!
Assessment of Thiamin /B1 status
RBC transketolase assay: measure RBC Transketolase activity, TPPbinding should be 85%
then add TPP should not shift
Riboflavin B2
riboFlavin/b2 = Fmn/ Fad active coenzyme forms
Redox cofactors, can participate in either one or 2 electorn redox rxns,
TCA cycle, and ETC, in FA b- oxidation
GSH reductase
Deficiecny energy metabolism, aminoacid metabolism and lipid metabolism, general and specific symptoms
Corners of mouth, phototherapy for neonatal jaundice breaks down riboflavin in infants so need B 2 supplementation, will affect RBC GSHr first
Niacin- B3
sources- dietary Niacin ( nicotinic acid in plants or nicotinomide in animal tissues)
Synthesized from tryptophan!
NAD, NADH - niacin NAD and NADPH
Glycolysis, TCA, FA b-oxidation, need NAD
ETC provides NAD
FAsynthesis and cholesterol synthesis need NADPH
HMP path gives NADPH
Niacin B3 deficiency
Pellagra- 4 Ds
Dermatitis, diarrhea, dementia, death
US
in dev countries- alcoholism
Hartnup disease- decreased Tryptophan for B3 synthesis
Pantothenic acid B5
PAN-availability, Widely distributed in nature and foods, deficiency is rare,
active forms- CoEnzyme A (CoASH, CoA), Acyl Carrier protein (ACP)
Biotin B7
food bound
Broadly distributed in many foods
Intestinal bacteria synthesize small amounts of biotin
Biotin bound to protein in foods and bound endogenously to carboxylase enzymes
Biotindidase releases biotin from dietary proteins and carboxylase enzymes for recycling of biotin
Holocarboxylase synthetase links biotin to carboxylase enzymes to obtain fully functional enzymes
Multiple carboxylase deficiency: autosomal recessive genetic defects in: Biotinidase gene, holocarboxylase synthetase gene
Many cases respond to high dose biotin therapy
Biotin B 7 Functions
Carboxylation reactions: Gluconeogenesis Pyruvate carboxylase
FA synthesis AcCoA carboxylase
BCAA (ile, val, leu) and odd chain fatty acid catabolism (Propionyl CoA carboxylase), (B- methylcrotonyl- CoA carboxylase (Leu)
Biotin B7 deficiency
Rare, Avidin in raw eggwhites binds dietary biotin making it unavailable
Rare autosomal recessive disorder- multiple carboxylase deficiecy, respond to biotin
Dry skin intractable seborrheic dermatitis
Pyridoxine- b6
active forms- PALP, PLP, PAMP, PMP
Proteins!
metabolic interconversions need Zinc, FMN- B2 riboflavin
transaminations- amino acid catabolism, non essential amino acid synthesis
histamine, NT synthesis
Homocysteine metabolism
homocystinuria type 1- due to genetic mutations in this enzyme, .5 of cases respond to high dose B6 (eye skeletal and CNS, vasculature, thromboembolism)- increased urine and plasma
VIT B6 deficiency
homocysteinemia, homocystinuria 1, increase in urinary homocystine, inc. plasma homocysteine, inc. plasma methionin, thromboembolism
B6 deficiency inducible by isoniazid, oral contraceptives