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Identification
HMDB Protein ID HMDBP00675
Secondary Accession Numbers
  • 5948
  • HMDBP02424
  • HMDBP04869
Name Glutaminase liver isoform, mitochondrial
Synonyms
  1. GLS
  2. L-glutaminase
  3. L-glutamine amidohydrolase
  4. SubName: L-glutaminase
Gene Name GLS2
Protein Type Enzyme
Biological Properties
General Function Involved in glutaminase activity
Specific Function Plays an important role in the regulation of glutamine catabolism. Promotes mitochondrial respiration and increases ATP generation in cells by catalyzing the synthesis of glutamate and alpha-ketoglutarate. Increases cellular anti-oxidant function via NADH and glutathione production. May play a role in preventing tumor proliferation.
Pathways
  • 2-Hydroxyglutric Aciduria (D And L Form)
  • 4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency
  • Alanine, aspartate and glutamate metabolism
  • Ammonia Recycling
  • Arginine and proline metabolism
  • Argininemia
  • Argininosuccinic Aciduria
  • Carbamoyl Phosphate Synthetase Deficiency
  • Citrullinemia Type I
  • D-Glutamine and D-glutamate metabolism
  • GABAergic synapse
  • Glutamate Metabolism
  • Glutamatergic synapse
  • Glutaminolysis and Cancer
  • Homocarnosinosis
  • Hyperinsulinism-Hyperammonemia Syndrome
  • Ornithine Transcarbamylase Deficiency (OTC Deficiency)
  • Proximal tubule bicarbonate reclamation
  • Succinic semialdehyde dehydrogenase deficiency
  • The oncogenic action of 2-hydroxyglutarate
  • The oncogenic action of D-2-hydroxyglutarate in Hydroxygluaricaciduria
  • The oncogenic action of L-2-hydroxyglutarate in Hydroxygluaricaciduria
  • Urea Cycle
  • Warburg Effect
Reactions
L-Glutamine + Water → L-Glutamic acid + Ammonia details
D-Glutamine + Water → D-Glutamic acid + Ammonia details
GO Classification
Biological Process
cellular nitrogen compound metabolic process
glutamine metabolic process
reactive oxygen species metabolic process
cellular amino acid biosynthetic process
regulation of apoptotic process
neurotransmitter secretion
glutamate secretion
Cellular Component
mitochondrial matrix
Function
catalytic activity
hydrolase activity
hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds
glutaminase activity
hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds, in linear amides
Molecular Function
glutaminase activity
Process
metabolic process
cellular metabolic process
glutamine metabolic process
glutamine family amino acid metabolic process
cellular amino acid and derivative metabolic process
cellular amino acid metabolic process
Cellular Location
  1. Cytoplasmic
  2. Mitochondrion
Gene Properties
Chromosome Location 12
Locus 12q13
SNPs GLS2
Gene Sequence
>1809 bp
ATGCGCTCCATGAAGGCTCTGCAGAAGGCCCTGAGCCGGGCTGGCAGTCACTGCGGGCGA
GGAGGCTGGGGTCACCCGAGCCGGAGCCCCCTCCTTGGCGGGGGCGTCCGGCACCACCTC
AGTGAGGCCGCGGCGCAGGGCAGAGAGACGCCACACAGCCACCAGCCGCAGCACCAGGAT
CATGATTCATCAGAAAGTGGCATGCTGTCCCGCCTGGGTGATTTGCTCTTTTACACTATT
GCTGAAGGACAGGAACGAACCCCTATCCACAAGTTCACCACTGCACTAAAGGCCACTGGA
CTGCAGACATCAGATCCTCGGCTCCGAGACTGCATGAGCGAGATGCACCGCGTGGTCCAA
GAGTCCAGTAGTGGTGGCCTCTTGGACCGAGATCTCTTCCGAAAGTGTGTGAGCAGCAGC
ATTGTGCTCCTGACCCAGGCATTCCGAAAGAAGTTTGTCATTCCTGATTTTGAGGAGTTC
ACGGGCCATGTGGATCGCATCTTTGAGGATGTCAAAGAGCTCACTGGAGGCAAAGTGGCA
GCCTACATCCCTCAGCTGGCCAAGTCAAACCCAGACCTGTGGGGTGTCTCCCTGTGCACT
GTGGATGGTCAACGGCACTCTGTGGGCCACACAAAGATCCCCTTCTGCCTGCAGTCCTGT
GTGAAGCCCCTCACCTATGCCATCTCCATAAGCACCCTAGGCACTGACTACGTGCACAAG
TTTGTGGGCAAAGAGCCAAGTGGCCTGCGCTACAACAAGCTCTCCCTCGATGAGGAAGGA
ATCCCCCATAACCCCATGGTCAATGCTGGTGCCATTGTTGTCAGCTCCCTGATCAAGATG
GACTGTAACAAAGCAGAGAAGTTTGATTTTGTGTTGCAGTATCTCAACAAAATGGCTGGG
AATGAATACATGGGTTTCAGCAATGCCACATTCCAGTCAGAGAAGGAAACAGGGGATCGG
AATTATGCCATCGGCTATTATCACGAGGAAAAGAAGTGCTTTCCTAAGGGGGTGGACATG
ATGGCTGCCCTTGATCTCTACTTCCAGCTGTGTTCTGTGGAGGTCACTTGTGAATCAGGC
AGTGTCATGGCAGCCACCCTCGCCAACGGTGGGATTTGCCCCATCACAGGCGAGAGTGTG
CTGAGTGCTGAAGCAGTGCGCAACACCCTCAGCCTCATGCATTCCTGCGGCATGTATGAC
TTCTCTGGCCAGTTTGCCTTCCACGTGGGCCTGCCAGCCAAGTCAGCTGTATCAGGAGCC
ATCCTCCTGGTGGTACCCAATGTCATGGGAATGATGTGCCTGTCACCCCCATTGGACAAG
CTGGGGAACAGCCATAGGGGGACCAGCTTCTGCCAGAAGTTGGTGTCTCTCTTCAATTTC
CACAACTATGACAACCTGAGGCACTGTGCTCGGAAGTTAGACCCACGGCGTGAAGGGGCA
GAAATTCGGAACAAGACTGTGGTCAACCTGTTATTTGCTGCCTATAGTGGCGATGTCTCA
GCTCTTCGAAGGTTTGCCTTGTCAGCCATGGATATGGAACAGAAAGACTATGACTCGCGC
ACAGCTCTGCATGTTGCTGCAGCTGAAGGACACATCGAAGTTGTTAAATTCCTGATCGAG
GCTTGCAAAGTGAATCCTTTTGCCAAGGACAGGTGGGGCAACATTCCCCTGGATGATGCT
GTGCAGTTCAACCATCTGGAGGTGGTCAAACTGCTTCAAGATTACCAGGACTCCTACACA
CTCTCTGAAACTCAGGCTGAGGCAGCAGCTGAGGCCCTGTCCAAAGAGAACTTAGAAAGC
ATGGTATGA
Protein Properties
Number of Residues 602
Molecular Weight 66322.225
Theoretical pI 7.301
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Glutaminase liver isoform, mitochondrial
MRSMKALQKALSRAGSHCGRGGWGHPSRSPLLGGGVRHHLSEAAAQGRETPHSHQPQHQD
HDSSESGMLSRLGDLLFYTIAEGQERIPIHKFTTALKATGLQTSDPRLRDCMSEMHRVVQ
ESSSGGLLDRDLFRKCVSSNIVLLTQAFRKKFVIPDFEEFTGHVDRIFEDVKELTGGKVA
AYIPQLAKSNPDLWGVSLCTVDGQRHSVGHTKIPFCLQSCVKPLTYAISISTLGTDYVHK
FVGKEPSGLRYNKLSLNEEGIPHNPMVNAGAIVVSSLIKMDCNKAEKFDFVLQYLNKMAG
NEYMGFSNATFQSEKETGDRNYAIGYYLKEKKCFPKGVDMMAALDLYFQLCSVEVTCESG
SVMAATLANGGICPITGESVLSAEAVRNTLSLMHSCGMYDFSGQFAFHVGLPAKSAVSGA
ILLVVPNVMGMMCLSPPLDKLGNSHRGTSFCQKLVSLFNFHNYDNLRHCARKLDPRREGA
EIRNKTVVNLLFAAYSGDVSALRRFALSAMDMEQKDYDSRTALHVAAAEGHIEVVKFLIE
ACKVNPFAKDRWGNIPLDDAVQFNHLEVVKLLQDYQDSYTLSETQAEAAAEALSKENLES
MV
GenBank ID Protein 6650606
UniProtKB/Swiss-Prot ID Q9UI32
UniProtKB/Swiss-Prot Entry Name GLSL_HUMAN
PDB IDs Not Available
GenBank Gene ID AF110330
GeneCard ID GLS2
GenAtlas ID GLS2
HGNC ID HGNC:29570
References
General References
  1. Gomez-Fabre PM, Aledo JC, Del Castillo-Olivares A, Alonso FJ, Nunez De Castro I, Campos JA, Marquez J: Molecular cloning, sequencing and expression studies of the human breast cancer cell glutaminase. Biochem J. 2000 Jan 15;345 Pt 2:365-75. [PubMed:10620514 ]
  2. Olalla L, Aledo JC, Bannenberg G, Marquez J: The C-terminus of human glutaminase L mediates association with PDZ domain-containing proteins. FEBS Lett. 2001 Jan 19;488(3):116-22. [PubMed:11163757 ]
  3. Perez-Gomez C, Mates JM, Gomez-Fabre PM, del Castillo-Olivares A, Alonso FJ, Marquez J: Genomic organization and transcriptional analysis of the human l-glutaminase gene. Biochem J. 2003 Mar 15;370(Pt 3):771-84. [PubMed:12444921 ]