TAZ; tafazzin (cardiomyopathy, dilated 3A (X-linked) endocardial fibroelastosis 2 Barth syndrome); HGNC: 11577; BTHS; CMD3A; EFE; EFE2; G4.5; XAP-2; OTTHUMP00000061673; cardiomyopathy, dilated 3A (X-linked); tafazzin; XX-FW83563B9.3; FLJ27390; Taz1; LVNC; Tafazzin; Tafazzin

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

100ug of dried peptide

Shipped at ambient temperature, store at -20 degree C

Manufactured in an ISO 9001:2008 Certified Laboratory.

Small volumes of TAZ blocking peptide 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.

25,836 Da

tafazzin

tafazzin

Tafazzin

EFE2; G4.5??[Similar Products]

TAZ_HUMAN

This gene encodes a protein that is expressed at high levels in cardiac and skeletal muscle. Mutations in this gene have been associated with a number of clinical disorders including Barth syndrome, dilated cardiomyopathy (DCM), hypertrophic DCM, endocardial fibroelastosis, and left ventricular noncompaction (LVNC). Multiple transcript variants encoding different isoforms have been described. A long form and a short form of each of these isoforms is produced; the short form lacks a hydrophobic leader sequence and may exist as a cytoplasmic protein rather than being membrane-bound. Other alternatively spliced transcripts have been described but the full-length nature of all these transcripts is not known. [provided by RefSeq, Jul 2008]

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: Transferase; Mitochondrial; Membrane protein, integral

Chromosomal Location of Human Ortholog: Xq28

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

Molecular Function: 1-acylglycerol-3-phosphate O-acyltransferase activity; 1-acylglycerophosphocholine O-acyltransferase activity; O-acyltransferase activity

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

Disease: Barth Syndrome

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