glycine cleavage system H protein (GCSH)

Function

• component of glycine cleavage system
• shuttles methylamine group of glycine from the P protein to the T protein

Cofactor: binds 1 lipoyl cofactor covalently

Structure

• belongs to the gcvH family
• contains 1 lipoyl-binding domain

Compartment

mitochondria

Pathology

• defects in GCSH are a cause of non-ketotic hyperglycinemia

More general terms

• mitochondrial protein
• protein subunit

Component of

• glycine cleavage system

Database

• UniProt: http://www.uniprot.org/uniprot/P23434.html
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=2653
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:2653
• OMIM: https://mirror.omim.org/entry/238330
• OMIM: https://mirror.omim.org/entry/605899