OPLAH - 5-oxoprolinase, ATP-hydrolysing Gene

Also Known as OPLA; OPLAHD; 5-Opase

Species: Homo sapiens

Gene Type: protein coding
Gene ID: 26873

About OPLAH

Cytogenetic location: 8q24.3 Genomic coordinates (GRCh38): 8:144,050,322-144,063,961 (from NCBI)

This gene has 4 transcripts (splice variants), 178 orthologues and is associated with 2 phenotypes. Broad expression in testis (RPKM 16.8), kidney (RPKM 6.8) and 20 other tissues.

Summary

The protein encoded by this gene acts as a homodimer, using ATP hydrolysis to catalyze the conversion of 5-oxo-L-proline to L-glutamate. Defects in this gene are a cause of 5-oxoprolinase deficiency (OPLAHD). [provided by RefSeq, Jun 2012]

OPLAH Products (1)

mRNA Protein Name
NM_017570.5 NP_060040.1 5-oxoprolinase
Molecular Function GO Annotation Evidence References Source
enables identical protein binding IPI
IPI: Inferred from physical interaction
32296183 GOA
enables protein binding IPI
IPI: Inferred from physical interaction
32296183 GOA
EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

OPLAH Protein Structure

Hydant_A_N

Hydant_A_N: Hydantoinase/oxoprolinase N-terminal region (10 - 212)

Hydantoinase_A

Hydantoinase_A: Hydantoinase/oxoprolinase (231 - 530)

Hydantoinase_B

Hydantoinase_B: Hydantoinase B/oxoprolinase (734 - 1255)

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  • 1288 a.a.
Protein Preferred Names Protein Names

5-oxoprolinase

  • 5-oxo-L-prolinase

OPLAH Protein-protein interaction Information

Type
Protein Name Protein ID Interactor Interactor Species Interactor ID Detection Method References
Intra
OPLAH O14841 POU6F2 Homo sapiens P78424 32296183
Intra
OPLAH O14841 POU6F2 Homo sapiens P78424 32296183
Intra
OPLAH O14841 POU6F2 Homo sapiens P78424 32296183
Intra
OPLAH O14841 ccsb orf id: 1345 Homo sapiens EBI-22333177 32296183
Intra
OPLAH O14841 TPK1 Homo sapiens Q9H3S4 32296183
Intra
OPLAH O14841 OPLAH Homo sapiens O14841 32296183
Intra
OPLAH O14841 TPK1 Homo sapiens Q9H3S4 32296183
Intra
OPLAH O14841 HOXA1 Homo sapiens P49639 21653829
Intra
OPLAH O14841 ccsb orf id: 1345 Homo sapiens EBI-22333177 32296183
Intra
OPLAH O14841 OPLAH Homo sapiens O14841 32296183
Intra
OPLAH O14841 OPLAH Homo sapiens O14841 32296183
Intra
OPLAH O14841 TPK1 Homo sapiens Q9H3S4 32296183
Cross: Cross-species interaction Intra: Intraspecies interaction

OPLAH Antibodies

Cat. No. Product Name Application Reactivity
HY-P810113 OPLAH Antibody (YA9457) WB, ICC/IF, IF-Tissue, IP, ELISA human, mouse, rat

Related Diseases

Diseases Alias
5-Oxoprolinase Deficiency
  • OPLAHD

  • Oxoprolinuria Due To 5-Oxoprolinase Deficiency

  • Oxoprolinuria Due To Oxoprolinase Deficiency

  • 5-Alpha-Oxoprolinase Deficiency

Glutathione Synthetase Deficiency
  • 5-Oxoprolinuria

  • Pyroglutamic Aciduria

  • Pyroglutamicaciduria

  • Glutathione Synthetase Deficiency With 5-Oxoprolinuria

  • GSSD

  • Oxoprolinase Deficiency

  • 5-Oxoprolinemia

  • Deficiency Of Glutathione Synthase

  • Deficiency Of Glutathione Synthetase

  • Pyroglutamic Acidemia

  • GSS DEFICIENCY

  • Gluthathione Synthetase Deficiency

  • 5-Oxoprolinase Deficiency

Tinea Favosa
  • Favus

Mend Syndrome
  • Male Ebp Disorder With Neurological Defects

  • MEND

  • Male Ebp Disorder With Neurologic Defects

Hawkinsinuria
  • 4-Alpha-Hydroxyphenylpyruvate Hydroxylase Deficiency

  • 4-Hppd Deficiency

  • 4-Hydroxyphenylpyruvic Acid Dioxygenase Deficiency

  • HWKS

  • HAWK

Metabolic Acidosis
Hypoinsulinemic Hypoglycemia With Hemihypertrophy
  • Hypoinsulinemic Hypoglycemia And Body Hemihypertrophy

  • HIHGHH

Cystinosis
  • Cystine Storage Disease

  • Cystine Diathesis

  • Cystine Disease

  • Cystinoses

  • Protein Defect Of Cystin Transport

  • Cystin Transport, Protein Defect Of

  • Nephropathic Cystinosis

  • Protein Defect Of Cystine Transport

Ck Syndrome
  • CKS

  • X-Linked Intellectual Disability-Microcephaly-Cortical Malformation-Thin Habitus Syndrome

  • Mental Retardation, X-Linked, With Thin Body Habitus And Cortical Malformation

Hemolytic Anemia
  • Anemia, Hemolytic

  • Anemia Hemolytic

  • Anaemia Due To Other Disorders Of Glutathione Metabolism

  • Chronic Non Spherocytic Anaemia

  • G6pd - [Glucose-6-Phosphate Dehydrogenase Deficiency] Anaemia

  • Anaemia Due To Glucose-6-Phosphate Dehydrogenase Deficiency

  • Glucose-6-Phosphate Dehydrogenase Deficiency With Anaemia

  • Glucose-6-Phosphate Dehydrogenase Deficiency Anaemia

  • Favism Anaemia

  • Haemolytic Anaemia Due Tog6pd Deficiency

  • Favism

  • Pentose Phosphate Pathway Disorder Anaemia

  • Anaemia Due To Pentose Phosphate Pathway Defect

Hypotonia-Cystinuria Syndrome
  • Cystinuria With Mitochondrial Disease

  • 2p21 Microdeletion Syndrome

  • HCS

  • Homozygous 2p16 Deletion Syndrome, Formerly

  • 2p21 Deletion Syndrome

  • Del(2)(P21)

  • Monosomy 2p21

  • Atypical Hypotonia-Cystinuria Syndrome

  • Atypical Hcs

Microcephaly
  • Microencephaly

  • Microcephalus

  • Microcephalic

  • Nanocephaly

  • Congenital Microcephaly

  • Brain Hypoplasia

  • Brain Nondevelopment

  • Cephalic Hypoplasia

  • Undeveloped Cerebrum

  • Undeveloped Brain

  • Micrencephalon

  • Micrencephaly

Diseases Alias
Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Orthologs Information

Species Symbol Source ID
Mus musculus OPLAH MGD MGI:1922725
Bos taurus OPLAH VGNC VGNC:53869
Felis catus OPLAH VGNC VGNC:68630
Canis familiaris OPLAH VGNC VGNC:49607
Rattus norvegicus OPLAH RGD RGD:620956
Macaca mulatta OPLAH VGNC VGNC:84096
Others OPLAH NCBI