| Primary Identifier | IPR034336 | Type | Family |
| Short Name | GDAP1 |
| description | GDAP1 family is a subfamily of the glutathione S-transferase (GST) family. Mutations of the GDAP1 gene cause Charcot-Marie-Tooth disease (CMT), one of the most frequent inherited peripheral neuropathy in humans []. GDAP1 is an integral, tail-anchored protein of the mitochondrial outer membrane (MOM) and the peroxisomal membrane, predominantly expressed in neural cells. The recombinant human GDAP1 has been shown to have specific GSH-conjugating activity in vitro, this activity is regulated by its hydrophobic domain 1 (HD1) []. This entry also includes GDAP1L1 (ganglioside-induced differentiation-associated protein 1-like 1), which is a paralogue of GDAP1 []. GDAP1L1 is capable of substituting for the loss of GDAP1 in the central nervous system of GDAP1-deficient mice [].Proteins in this subfamily contains the GST-N and the GST-C domains, and an extended interdomain linker which may adopt two additional α-helices []. |
