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23 Cards in this Set

  • Front
  • Back

Essential amino Acids

AA's that must be introduced through the diet

Non essential amino Acids

AA's that don't to be introduced through diet

Glutogentic AA's

Produce substrate for glucose when canabolized

Ketogeic AA's

Produce Acetyl CoA or Acetoacetate when canabolized

Pyridoxal Phosphate (PLP or B6)

transanimation and transulfuration of AA's

Folate

Carbon Donor

Cobalamin (b12)

Conversions of:


- Homocystine to Methionine - Add Methyl


- Methylmanyl CoA to Succinyl CoA - Rearange mollecule


- Folate Cycle

Tetrahydrobiperin (BH4)

Used to convert:


- Phenylalanine to Tyrosine


- Tryptophan to seratonin


- Tyrosine to dopamine

Strictly ketogenic amino Acids


- Intermediate

Lysine


Leucine


Acetyl CoA

Autozomal

22 pairs of genes not sex genes


X or Y linkes

1 pair of genes sex genes

Wild type allele

Most likely to be seen in nature


Designated by (+)

how folate and SAM work together

Folate methylates B12


B12 Methylates methionine


Methionine becomes SAM


SAM converts products

Maple Syrup Disease

After branched chain amino acids are demainated there is a breakdown in the decarboxylation of the chain

Branched chain amino acids

Leucine, Isoleucine, Valine

Thyamine

Cofactor required for branched chain amino acids


Cofactor required for Alpa keto acid dehydrogenase

Thyamine

Causes of phenylketonourea

- Deficiency in phenylalanine hydrogenase


- Lack of Folate

Phenylalaine hydrogenase

Converts phenylalaine to tyrosine

homocysene, Cysteine, Methionine cycle

homocysteine can


1. Be methylated by folate to methionine by methionine synthase with b12 as cofactor


2. Trassulfurated by by cystathione beta synthase to cystene with b6 cofactor

cystathione beta synthase

Transulfuration of homocystene to cystene


- Requires B6

Methotrexate

inhibits reconstitution of folate

5-florouracil

unhibits enzyme that converts dUMP to dTDP