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===='''Introduction'''====
===='''Introduction'''====
Ornithine transcarbamoylase (OTC) is an enzyme that catalyzes the reaction between carbamoyl phosphate and ornithine to form citrulline and phosphate. In plants and microbes, OTC is involved in arginine biosynthesis, but in mammals it is located in the mitochondria and is part of the urea cycle.<ref>http://en.wikipedia.org/wiki/Ornithine_transcarbamylase</ref>
Ornithine transcarbamoylase (OTC) is an enzyme that catalyzes the reaction between carbamoyl phosphate and ornithine to form citrulline and phosphate. In plants and microbes, OTC is involved in arginine biosynthesis, but in mammals it is located in the mitochondria and is part of the urea cycle.<ref>http://en.wikipedia.org/wiki/Ornithine_transcarbamylase</ref>
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OTC is often associated with Ornithine transcarbamoylase deficiency (OTCD). OTCD is a common urea cycle disorder, and it is a genetic disorder which results in a mutated and ineffective form of the enzyme OTC.
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OTC is often associated with Ornithine transcarbamoylase deficiency (OTCD). OTCD is a common urea cycle disorder, and it is a genetic disorder which results in a mutated and ineffective form of the enzyme OTC. The gene is located on the short arm of chromosome X (Xp21.1). The gene is located in the Watson (plus) strand and is 68,968 bases in length. The encoded protein is 354 amino acids long with a predicted molecular weight of 39.935 kiloDaltons. The protein is located in the mitochondrial matrix.<ref>http://en.wikipedia.org/wiki/Ornithine_transcarbamylase</ref>

Revision as of 00:57, 14 November 2012

This Sandbox is Reserved from 30/08/2012, through 01/02/2013 for use in the course "Proteins and Molecular Mechanisms" taught by Robert B. Rose at the North Carolina State University, Raleigh, NC USA. This reservation includes Sandbox Reserved 636 through Sandbox Reserved 685.
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Ornithine Transcarbamoylase

Introduction

Ornithine transcarbamoylase (OTC) is an enzyme that catalyzes the reaction between carbamoyl phosphate and ornithine to form citrulline and phosphate. In plants and microbes, OTC is involved in arginine biosynthesis, but in mammals it is located in the mitochondria and is part of the urea cycle.[1] OTC is often associated with Ornithine transcarbamoylase deficiency (OTCD). OTCD is a common urea cycle disorder, and it is a genetic disorder which results in a mutated and ineffective form of the enzyme OTC. The gene is located on the short arm of chromosome X (Xp21.1). The gene is located in the Watson (plus) strand and is 68,968 bases in length. The encoded protein is 354 amino acids long with a predicted molecular weight of 39.935 kiloDaltons. The protein is located in the mitochondrial matrix.[2]



Structure

OTC is a trimer. The monomer unit has a CP-binding domain and an amino acid-binding domain. Each of the two discrete substrate-binding domains (SBDs) have an α/β topology with a central β-pleated sheet embedded in flanking α-helices. The active sites are located at the interface between the protein monomers.[3]

References:
  1. ↑ http://en.wikipedia.org/wiki/Ornithine_transcarbamylase
  2. ↑ http://en.wikipedia.org/wiki/Ornithine_transcarbamylase
  3. ↑ http://en.wikipedia.org/wiki/Ornithine_transcarbamylase
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