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Gamma glutamyl cycle

Gamma glutamyl cycle and specific transporters for intestinal Amino Aci uptake

QuestionAnswer
Dibasic Amino Acid Deficiency. Autosomal recessive.Findings: Cystine kidney stones, renal failure (rare), lysinuria, argininuria and ornithininuria Cystinuria
Why is there no amino acid deficiency in the patient with Cystinuria? Gamma Glutamyl Cycle imports dibasic amino acids effectively.
What arre the two mechanisms for intestinal amino acid uptake? Specific amono acid transporters and the Gamma Glutamyl Cycle.
What are the essential Amino acids? Use the private tim hall to answer. Phenylalanine, valine, tryptophan, thereonine, isoleucine, metheonine, histidine, arginine, leucine,lysine
Exception to the Essential amino acids: ARGININE is not esential to ___, and can be synthesized from____ . older adults,glutamate
Exception to the Essential amino acids: CYSTEINE is essential in ___ children
The amono acids transporters are ___ gradient dependent Na+
True or False: Cysteinuria is WELL-CONTROLLED BY A DIET LOW IN CYSTEINE True
What is the disulfide dimer of Cysteine Cystine
Large Neutral Amino acid Transporter defect in intestinal mucosa and renal tubules. With pellagra like symptoms, cerebellar ataxia and mild Tryptophan deficiency. Hartnup disease
What is pellagra? Niacin(vit B3) deficiency,
What are the pellegra like symptoms? Triple D DERMATITIS, DIARRHEA, DEMENTIA
What should be the treatment of Hartnup? Diet hight in tryptophan and niacin
Large neutral amino acid transporter for which amino acids? ILE,LEU,VAL,MET,PHE,TYR,TRP
Dibasic amino acid transporter for which amino acids? HIS,ARG,LYS,CysTINE
Gamma glutamyl cycle is dependent on what amino? Glutathione
____ is needed by RBS in its oxidized form to protect the cell against free radicals and oxidizing agents such as flava beans, sulfonamides, primaquines, etc. Glutathione
GGT is a transmembrane protein need for the transport of amino acids to the lumen of the enterocyte. It does not like to transport PRO and TRP so much. When it transports an amnino acid towards the lumen it uses Gluthathione to produce what? Glutamyl-amino acid + Cys-Gly
GGC is the enzyme that frees the amino acid from the glutamate part and also produces ___ an heterocyclic amino acid 5-oxoproline
5 oxoprolinase (5OP) catalized the reaction of 5 oxoproline to ____. And requires the spending of 1 ___ molecule. Glutamate, ATP
5 OP is the _________ enzyme rate limiting
Catalizes the reaction from glutamate to Glu-cys. In other wors adds the Cys to glutamate. Requires ATP GCS
Catalizes the reaction of Glu-cys to Gluthathione. Requires ATP GS
Gluthathione normally regulates the Gamma Glutamyl cycle by feedback inhibition on___ GCS
How many molecules of ATP are need for one turn of the Cycle? 3
Increased, 5 oxoprolinuria in urine, high anion gap metabolic acidosis, hemolytic anemia and defective leucocyte function are clinical findings of what autosomal recessive disease? Oxoprolinuria
What is the enzyme in the gamma glutamyl cycle that frees the amino acid from glutamate? GGC
Created by: rcraquel