Conserved Protein Domain Family
GST_N_GDAP1

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cd03052: GST_N_GDAP1 
GST_N family, Ganglioside-induced differentiation-associated protein 1 (GDAP1) subfamily; GDAP1 was originally identified as a highly expressed gene at the differentiated stage of GD3 synthase-transfected cells. More recently, mutations in GDAP1 have been reported to cause both axonal and demyelinating autosomal-recessive Charcot-Marie-Tooth (CMT) type 4A neuropathy. CMT is characterized by slow and progressive weakness and atrophy of muscles. Sequence analysis of GDAP1 shows similarities and differences with GSTs; it appears to contain both N-terminal TRX-fold and C-terminal alpha helical domains of GSTs, however, it also contains additional C-terminal transmembrane domains unlike GSTs. GDAP1 is mainly expressed in neuronal cells and is localized in the mitochondria through its transmembrane domains. It does not exhibit GST activity using standard substrates.
Statistics
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PSSM-Id: 239350
Aligned: 6 rows
Threshold Bit Score: 149.233
Created: 9-Mar-2005
Updated: 2-Oct-2020
Structure
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Aligned Rows:
 
Feature 1:putative GSH binding site (G-site) [chemical binding site]
Evidence:
  • Comment:The GST active site is composed of a GSH binding site (G-site), common to all GSTs, and a xenobiotic binding site (H-site), which varies between different classes and isotypes. Residues from the N-terminal TRX-fold domain form the G-site while the H-site is comprised mainly of residues from the C-terminal alpha helical domain.
  • Comment:Based on similarity with other GST family members.

Sequence Alignment
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Format: Row Display: Color Bits: Type Selection:
Feature 1               #                                       ###          ##        
Q8TB36     26 LILYHWTHSFSSQKVRLVIAEKALKCEGHDVSLPLSEHNEPWFMRLNSTGEVPVLIHGENIICEATQIIDYLE 98  human
XP_418303  11 LVLYHWTQSFSSQKVRLAIAEKALTCEEHDVNLPLSEHNEPWFMRLNASGEVPVLIHGENIICEATQIIDYLE 83  chicken
CAE17616   40 LILYHWTQSFSSQKVRLAIAEKGLQCEDYDVSLPLSEHNEPWFMRLNPTGEVPVLVHDNHVICDPTQIMDYLE 112 zebrafish
Q96MZ0     47 LVLYHWTQSFSSQKVRLVIAEKGLVCEERDVSLPQSEHKEPWFMRLNLGEEVPVIIHRDNIISDYDQIIDYVE 119 human
XP_708880  48 LVLYHWTQSFSSQKVRLVINEKGLLCEERDVSLPLTEQKEPWFMRLNLGEEVPVFIHGDTIVSDYNQIIDYIE 120 zebrafish
CAF98017   39 LTLYHWTQSFQLAEGAFGDTEKGLRCEEYDVSLPLSEHNEPWFMHLNPTGEVPVLVHGGRVICDPTQIVDYLE 111 Tetraodon nigroviridis

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