Hmdb loader
Identification
HMDB Protein ID HMDBP00812
Secondary Accession Numbers
  • 6092
  • HMDBP05975
Name Hydroxyacylglutathione hydrolase, mitochondrial
Synonyms
  1. Glx II
  2. Glyoxalase II
Gene Name HAGH
Protein Type Unknown
Biological Properties
General Function Involved in hydrolase activity
Specific Function Thiolesterase that catalyzes the hydrolysis of S-D-lactoyl-glutathione to form glutathione and D-lactic acid.
Pathways
  • Leigh Syndrome
  • methylglyoxal degradation
  • Primary hyperoxaluria II, PH2
  • Pyruvaldehyde Degradation
  • Pyruvate Decarboxylase E1 Component Deficiency (PDHE1 Deficiency)
  • Pyruvate Dehydrogenase Complex Deficiency
  • Pyruvate kinase deficiency
  • Pyruvate metabolism
  • Pyruvate metabolism
Reactions
S-(2-hydroxyacyl)glutathione + Water → Glutathione + a 2-hydroxy carboxylate details
S-Lactoylglutathione + Water → Glutathione + D-Lactic acid details
GO Classification
Biological Process
glutathione biosynthetic process
carbohydrate metabolic process
Cellular Component
mitochondrial matrix
cytoplasm
Function
ion binding
cation binding
metal ion binding
hydrolase activity, acting on ester bonds
binding
catalytic activity
hydrolase activity
transition metal ion binding
zinc ion binding
hydroxyacylglutathione hydrolase activity
thiolester hydrolase activity
Molecular Function
metal ion binding
zinc ion binding
hydroxyacylglutathione hydrolase activity
Cellular Location
  1. Isoform 2:Cytoplasm
Gene Properties
Chromosome Location 16
Locus 16p13.3
SNPs HAGH
Gene Sequence
>927 bp
ATGGTGGTGGGCCGAGGGCTGCTCGGCCGCCGCAGCCTCGCCGCGCTGGGAGCCGCCTGC
GCCCGCCGAGGCCTCGGTCCAGCCCTGCTGGGAGTTTTCTGCCACACAGATTTGCGGAAG
AACCTGACCGTGGACGAGGGCACCATGAAGGTAGAGGTGCTGCCTGCCCTGACCGACAAC
TACATGTACCTGGTCATTGATGATGAGACCAAGGAGGCTGCCATTGTGGATCCGGTGCAG
CCCCAGAAGGTCGTGGACGCGGCGAGAAAGCACGGGGTGAAACTGACCACAGTGCTCACC
ACCCACCACCACTGGGACCATGCTGGCGGGAATGAGAAACTGGTCAAGCTGGAGTCGGGA
CTGAAGGTGTACGGGGGTGACGACCGTATCGGGGCCCTGACTCACAAGATCACTCACCTG
TCCACACTGCAGGTGGGGTCTCTGAACGTCAAGTGCCTGGCGACCCCGTGCCACACTTCA
GGACACATTTGTTACTTCGTGAGCAAGCCCGGAGGCTCGGAGCCCCCTGCCGTGTTCACA
GGTGACACCTTGTTTGTGGCTGGCTGCGGGAAGTTCTATGAAGGGACTGCGGATGAGATG
TGTAAAGCTCTGCTGGAGGTCTTGGGCCGGCTCCCCCCGGACACAAGAGTCTACTGTGGC
CACGAGTACACCATCAACAACCTCAAGTTTGCACGCCACGTGGAGCCCGGCAATGCCGCC
ATCCGGGAGAAGCTGGCCTGGGCCAAGGAGAAGTACAGCATCGGGGAGCCCACAGTGCCA
TCCACCCTGGCAGAGGAGTTTACCTACAACCCCTTCATGAGAGTGAGGGAGAAGACGGTG
CAGCAGCACGCAGGTGAGACGGACCCGGTGACCACCATGCGGGCCGTGCGCAGGGAGAAG
GACCAGTTCAAGATGCCCCGGGACTGA
Protein Properties
Number of Residues 308
Molecular Weight 28859.855
Theoretical pI 7.323
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Hydroxyacylglutathione hydrolase, mitochondrial
MVVGRGLLGRRSLAALGAACARRGLGPALLGVFCHTDLRKNLTVDEGTMKVEVLPALTDN
YMYLVIDDETKEAAIVDPVQPQKVVDAARKHGVKLTTVLTTHHHWDHAGGNEKLVKLESG
LKVYGGDDRIGALTHKITHLSTLQVGSLNVKCLATPCHTSGHICYFVSKPGGSEPPAVFT
GDTLFVAGCGKFYEGTADEMCKALLEVLGRLPPDTRVYCGHEYTINNLKFARHVEPGNAA
IREKLAWAKEKYSIGEPTVPSTLAEEFTYNPFMRVREKTVQQHAGETDPVTTMRAVRREK
DQFKMPRD
GenBank ID Protein 94538322
UniProtKB/Swiss-Prot ID Q16775
UniProtKB/Swiss-Prot Entry Name GLO2_HUMAN
PDB IDs
GenBank Gene ID NM_005326.4
GeneCard ID HAGH
GenAtlas ID HAGH
HGNC ID HGNC:4805
References
General References
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