The enzyme system for cleavage of glycine (glycine cleavage system) is composed of four mitochondrial protein components: P protein, H protein, T protein, and L protein. GCSH is the H protein, which transfers the methylamine group of glycine from the P protein to the T protein. Defects in this gene are a cause of nonketotic hyperglycinemia (NKH).
Source:
Recombinant corresponding to aa49-173 from human GCSH, fused to His-tag at N-terminal expressed in E.coli.
AA Sequence:
MGSSHHHHHH SSGLVPRGSH MGSMSVRKFT EKHEWVTTEN GIGTVGISNF AQEALGDVVY CSLPEVGTKL NKQDEFGALE SVKAASELYS PLSGEVTEIN EALAENPGLV NKSCYEDGWL IKMTLSNPSE LDELMSEEAY EKYIKSIEE
Molecular Weight:
~16.4kD (MALDI-TOF)
Storage and Stability:
May be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.