Biochem: Vitamins Flashcards
Enzymes that have biotin as a co-factor
- pyruvate carboxylase (gluconeogenesis)
- acetyl CoA carboxylase (FA synthesis)
- propionyl CoA carboxylase (odd-carbon FA, val, Met, Ile, Thr)??
Biotin Deficiency:
- MCC
- symptoms
- MCC (rare): excessive consumption of raw eggs (contain avidin, a biotin-binding protein and prevents its absorption)
- Symptoms: alopecia (hair loss), bowel inflammation, muscle pain
Thiamine (vitamin B1) deficiency
- MCC
- symptoms
- In thiamine pyrophosphate (TPP) = a cofactor for several dehydrogenase enzyme rxns.*
- MCC: alcoholism (alcohol interferes with absorption)
- Wernicke (ataxia, nystagmus, ophthalmoplegia (wide-eyed, can’t follow light)
- korsakoff (confabulation, psychosis)
- wet beri beri (high output cardiac failure)
- dry (early) beri beri (cardiac failure without fluid retention)
*beri beri = heart failure due to Vit B1 deficiency
What enzymes require Vitamin B1 (Thiamine) as a cofactor?
- In thiamine pyrophosphate (TPP) = a cofactor for several dehydrogenase enzyme rxns.*
- pyruvate dehydrogenase (PDH?)
- alpha-ketoglutarate dehydrogenase (TCA cycle)
- transketolase (HMP shunt)
- branched chain ketoacid dehydrogenase (metabolism of valine, isoleucine and leucine)
Vitamin D Toxicity
- excess Vit D can be stored in liver as 25-hydroxycholecalciferol
- excess Vit D can promote intestinal absorption of calcium and phosphate
- results in hypercalcemia
- direct result of excess Vit D = same as Vit D deficiency
- Hypercalcemia can impair renal function, and early signs include polyuria, polydipsia, and nocturne
- prolonged hypercalcemia can result in calcium deposition in soft tissues (esp kidney), causing irreversible kidney damage
Vitamin A (carotene)
- Vit A is converted to several active forms in the body associated with 2 important functions:
1. maintenance of healthy epithelium and 2. vision (retinal) - important function: retinoid acid and retinol act as growth regulators (bind intracellular receptors (zinc-finger proteins) to regulate transcription), especially in epithelium.
- retinal is important in rod and cone cells for vision
- 3 Vit A structures w different functional groups at C1:
- Retinol: hydroxyl
- retinoid acid: carboxyl
- retinal: aldehyde
Vitamin D (cholecalciferol)
important function: in response to hypocalcemia, helps normalize serum calcium levels
Vitamin B3
Niacin
- remember, Niacin = NAD(H), NADP(H)
- involved in many pathways, because cofactor for all dehydrogenase (bc NAD)
Vitamin K (menaquinone, bacteria; phytoquinone, plants)
Important functions: carboxylation of glutamic acid residues in many Ca2+ binding proteins
- importantly coagulation factors 2, 7, 9 and 10 as well as protein C and protein S
Vitamin E (alpha-tocopherol)
antioxidant in the lipid phase. protects membrane lipids from preoccupation
What is the cofactor for branched chain ketoacid dehydrogenase?
Thiamine B1
Symptoms of Vitamin D deficiency
- bone demineralization
- rickets (children): deformities of legs and other developing bones. muscle weakness common
- osteomalacia (adults, after epiphyseal fusion): less deformity than rickets. may present as bone pain and muscle weakness
Causes of Vitamin D Deficiency
- insufficient sunlight
- inadequate fortified foods (milk)
- end-stage renal disease (renal osteodystrophy)
What is the cofactor for alpha-ketoglutarate dehydrogenase?
Thiamine (B1)
Symptoms of Vitamin A deficiency
- night blindness
- keratinized squamous epithelium
- xerophthalmia, bitot spots
- keratomalacia, blindless
- follicular hyperkeratosis
- alopecia
Causes of Vitamin A deficiency
- fat malabsorption
- fat-free diets
Vitamin B2
Riboflavin (FAD, FMN)
Symptoms of Vitamin E deficiency
- hemolytic anemia
- acanthocytosis
- peripheral neuropathy
- ataxia
- retinitis pigmentosum
Causes of Vitamin E deficiency
- fat malabsorption
- premature infants
What is the cofactor for propionyl CoA carboxylase
biotin
pyruvate carboxylase is involved in what pathway?
gluconeogenesis
acetyl CoA carboxylase is involved in what pathway?
Fatty acid synthesis (carboxylation)
propionyl CoA carboxylase is involved in what pathway?
(odd-carbon FA, val, Met, Ile, Thr)??
What is the cofactor for acetyl CoA carboxylase
biotin
transketolase is involved in what pathway?
- HMP shunt
- this is the key enzyme involved in Wernicke Korsakoff syndrome
branched chain ketoacid dehydrogenase is involved in what pathway?
metabolism of valine isoleucine and leucine
A/e of isotretinoin
Teratogenic (malformation of the craniofacial, cardiac, thymic and CNS structures)
What is the cofactor for pyruvate carboxylase?
biotin
alpha-ketoglutarate dehydrogenase is involved in what pathway?
TCA cycle, carrier of FA
What is the cofactor for pyruvate dehydrogenase?
Thiamine (B1)
Vitamin B6
Pyridoxine (has an aldehyde!)
What is Pellagra?
- Vitamin B3 (niacin) deficiency
- Sx: Diarrhea, Dementia, Dermatitis, Death (if not treated)
- may also be related to deficiency of tryptophan (corn major dietary stable) which supplies a portion of the niacin requirement
Vitamin B12
Cyanocobalamin
What is the cofactor for transketolase?
Thiamine (B1)
Folic Acid deficiency
- MCC
- symptoms
- MCC: alcoholics and pregnancy (body stores depleted in 3 months)
- homocystinemia with risk of DVT and atherosclerosis
- megaloblastic (macrocytic) anemia
- deficiency in early pregnancy causes neural tube defects
lipid-soluble vitamins
- A (carotene)
- D (cholecalciferol)
- E (alpha-tocopherol)
- K (menaquinone, bacteria; phytoquinone, plants)
what enzymes require folic acid as a cofactor?
- thymidylate synthase (thymidine (pyrimidine) synthesis)
- enzymes in purine synthesis (don’t need to know specifics)
- Remember, Folic acid = THF
How does folic acid deficiency differ from Vitamin B12 deficiency?
Vit B12 deficiency has peripheral neuropathy
What condition has pellagra-like symptoms because of decreased tryptophan?
Hartnup
What enzymes have Vit B12 (cyanocobalamin) as a cofactor?
- homocysteine methyltransferase (methionine, SAM)
- methylmanoyl CoA mutase (VOMIT pathway: val, Met, Ile, Thr, Odd-carbon FA)
Ascorbate (C) deficiency:
- MCC
- symptoms
- MCC: diet deficient in citrus fruits and green veggies
- Scurvy: microcytic, hypochromic, Fe deficiency anemia
- Scurvy Sx: poor wound healing, easy bruising (perifollicular hemorrhage), bleeding gums, increased bleeding time, painful glossitis, anemia
Which pathway is thymidylate synthesis in?
thymidine (pyrimidine) synthesis