SG ID

SG00000293 

UniProt Accession

Q8MR13_DROME;Q8MR13;  

Theoretical PI

6.67  

Molecular Weight

42693 Da  

Genbank Nucleotide ID

AY122192;  

Genbank Protein ID

AAM52704.1; 

Gene Name

iPLA2-VIA 

Gene Synonyms/Alias

ORFNames=CG6718 

Protein Name

 

Protein Synonyms/Alias

SubName: LD44515p 

Drosophila melanogaster (Fruit fly) 

7227 

Temporarily unavailable 

The information of related literatures

1. A. Malhotra, I. Edelman-Novemsky, Y. Xu, H. Plesken, J. Ma, M. Schlame and M. Ren (2009) Role of calcium-independent phospholipase A2 in the pathogenesis of Barth syndrome. Proc Natl Acad Sci U S A 106(7): 2337-41. 

Abstract
Quantitative and qualitative alterations of mitochondrial cardiolipin have been implicated in the pathogenesis of Barth syndrome, an X-linked cardioskeletal myopathy caused by a deficiency in tafazzin, an enzyme in the cardiolipin remodeling pathway. We have generated and previously reported a tafazzin-deficient Drosophila model of Barth syndrome that is characterized by low cardiolipin concentration, abnormal cardiolipin fatty acyl composition, abnormal mitochondria, and poor motor function. Here, we first show that tafazzin deficiency in Drosophila disrupts the final stage of spermatogenesis, spermatid individualization, and causes male sterility. This phenotype can be genetically suppressed by inactivation of the gene encoding a calcium-independent phospholipase A(2), iPLA2-VIA, which also prevents cardiolipin depletion/monolysocardiolipin accumulation, although in wild-type flies inactivation of the iPLA2-VIA does not affect the molecular composition of cardiolipin. Furthermore, we show that treatment of Barth syndrome patients' lymphoblasts in tissue culture with the iPLA(2) inhibitor, bromoenol lactone, partially restores their cardiolipin homeostasis. Taken together, these findings establish a causal role of cardiolipin deficiency in the pathogenesis of Barth syndrome and identify iPLA2-VIA as an important enzyme in cardiolipin deacylation, and as a potential target for therapeutic intervention. PMID: [19164547] 

Figures for illustrating the function of this protein/gene

Function

 

Subcellular Location

 

Tissue Specificity

 

Gene Ontology

GO ID	GO term	Evidence
GO:0016787	F:hydrolase activity	IEA:UniProtKB-KW.
GO:0006629	P:lipid metabolic process	IEA:InterPro.

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Interpro

IPR016035;    Acyl_Trfase/lysoPLipase.
IPR002641;    Patatin/PLipase_A2-rel.
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Pfam

PF01734;    Patatin;    1.
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SMART

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PROSITE

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PRINTS

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Created Date

18-Oct-2012 

Record Type

Experiment identified 

Protein sequence Annotation

Nucleotide Sequence

Length: 2331 bp   Go to nucleotide: FASTA

Protein Sequence

Length: 386 bp   Go to amino acid: FASTA

The verified Protein-Protein interaction information

UniProt

Gene Symbol

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Other Protein-Protein interaction resources

String database  

UniProt

Gene Symbol

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View Microarray data

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