For Research Use Only. Not for use in diagnostic procedures.

Chromosome: X; NC_000086.7 (74281882..74290151). Location: X C1-C3; X 37.95 cM

E Coli

> 95%

Supplied as lyophilized form in PBS,pH7.4, containing 5% sucrose, 0.01% sarcosyl.

Avoid repeated freeze/thaw cycles. Store at 2-8 degree C for one month. Aliquot and store at -80 degree C for 12 months.
Stability Test: The thermal stability is described by the loss rate of the targetprotein. The loss rate was determined by accelerated thermal degradation test,that is, incubate the protein at 37 degree C for 48h, and no obvious degradation andprecipitation were observed. (Referring from China Biological Products Standard,which was calculated by the Arrhenius equation.) The loss of this protein is lessthan 5% within the expiration date under appropriate storage condition.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

Small volumes of TAZ recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

About the Marker: Effective Size Range: 10kDa to 70kDa.
Protein bands: 10kDa, 14kDa, 18kDa, 22kDa, 26kDa, 33kDa, 44kDa and70kDa.
Double intensity bands: The 26kDa, 18kDa, 10kDa bands are at doubleintensity to make location and size approximation of proteins of interestquick and easy.
Ready-to-use: No need to heat, dilute or add reducing agents before use.

SDS-PAGE, Western Blot (WB), ELISA (EIA), Immunoprecipitation (IP)

26.2kDa

tafazzin

tafazzin

Tafazzin

I7HJS2_MOUSE

This gene encodes a mitochondrial phospholipid-lysophospholipid transacylase necessary for normal composition and content of cardiolipin. In humans, mutations of this gene result in Barth syndrome, most often characterized by cardioskeletal myopathy, neutropenia and abnormal mitochondria. This gene is distinct from the gene encoding transcriptional coactivator with PDZ binding motif. Both genes share the gene symbol Taz. Multiple transcript variants encoding different isoforms have been described. [provided by RefSeq, Mar 2010]

tafazzin: Some isoforms may be involved in cardiolipin (CL) metabolism. Defects in TAZ are the cause of Barth syndrome (BTHS). An X-linked disease characterized by dilated cardiomyopathy with endocardial fibroelastosis, a predominantly proximal skeletal myopathy, growth retardation, neutropenia, and organic aciduria, particularly excess of 3-methylglutaconic acid. Additional features include hypertrophic cardiomyopathy, isolated left ventricular non-compaction, ventricular arrhythmia, motor delay, poor appetite, fatigue and exercise intolerance, hypoglycemia, lactic acidosis, hyperammonemia, and dramatic late catch-up growth after growth delay throughout childhood. Belongs to the taffazin family. 9 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, integral; Mitochondrial; Transferase

Cellular Component: membrane; mitochondrion; mitochondrial membrane; intrinsic to membrane; nucleus; cytosol

Molecular Function: 1-acylglycerophosphocholine O-acyltransferase activity; O-acyltransferase activity

Biological Process: cardiolipin biosynthetic process; cardiac muscle development; inner mitochondrial membrane organization and biogenesis; mitochondrial respiratory chain complex I assembly; skeletal muscle development; cristae formation; muscle contraction; heart development; phosphatidylglycerol metabolic process; hemopoiesis; cardiac muscle contraction; organelle ATP synthesis coupled electron transport

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