SpermatogenesisOnline 1.0
 

Tag Content
SG ID
SG00000260 
UniProt Accession
Theoretical PI
9.35  
Molecular Weight
43016 Da  
Genbank Nucleotide ID
Genbank Protein ID
Gene Name
Taz 
Gene Synonyms/Alias
ORFNames=CG8766 
Protein Name
Tafazzin homolog 
Protein Synonyms/Alias
dTAZ 
Organism
Drosophila melanogaster (Fruit fly) 
NCBI Taxonomy ID
7227 
Chromosome Location
chr:2R;8640531-8643915;-1
View in Ensembl genome browser  
Function in Stage
Function in Cell Type
Description
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] 

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Figures for illustrating the function of this protein/gene
Ref: 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. PMID: [19164547]
Function
Essential for the formation of normal cardiolipin (CL).Essential for the final stage of spermatogenesis, spermatidindividualization. 
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Subcellular Location
Membrane; Single-pass membrane protein (Bysimilarity). Note=Isoforms with hydrophobic N-terminus are thoughtto be membrane-anchored (By similarity). 
Tissue Specificity
 
Gene Ontology
GO IDGO termEvidence
GO:0016021 C:integral to membrane NAS:UniProtKB.
GO:0005739 C:mitochondrion IDA:FlyBase.
GO:0032577 F:phosphatidylcholine:cardiolipin O-linoleoyltransferase activity IDA:FlyBase.
GO:0032049 P:cardiolipin biosynthetic process IMP:UniProtKB.
GO:0007006 P:mitochondrial membrane organization IDA:FlyBase.
GO:0007291 P:sperm individualization IMP:UniProtKB.
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Interpro
IPR002123;    Acyltransferase.
IPR000872;    Tafazzin.
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Pfam
PF01553;    Acyltransferase;    1.
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SMART
SM00563;    PlsC;    1.
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PROSITE
PRINTS
PR00979;    TAFAZZIN.;   
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Created Date
18-Oct-2012 
Record Type
Experiment identified 
Protein sequence Annotation
CHAIN         1    378       Tafazzin homolog.
                             /FTId=PRO_0000220933.
TRANSMEM    138    158       Helical; (Potential).
REGION      243    283       Hydrophilic (By similarity).
VAR_SEQ       1    100       Missing (in isoform B).
                             /FTId=VSP_004450.
VAR_SEQ       1     41       MFMVVCSNLRRPGHVGAASAARNINWLISEGYTPPIRAMAR
                             -> M (in isoform C).
                             /FTId=VSP_007017.
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Nucleotide Sequence
Length: 1577 bp   Go to nucleotide: FASTA
Protein Sequence
Length: 378 bp   Go to amino acid: FASTA
The verified Protein-Protein interaction information
UniProt
 Gene Symbol Ref Databases
pAbpBioGRID 
CG15530BioGRID 
CG6425BioGRID 
Other Protein-Protein interaction resources
String database  
View Microarray data
Temporarily unavailable 
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