antibody

Anti-Tafazzin/TAZ antibody

MBS176048

0.1 mg

280 USD

polyclonal

rabbit

nonconjugated

human, rat

Antibody

Anti-Tafazzin/TAZ antibody

[Tafazzin/TAZ]

[Tafazzin; tafazzin; Barth syndrome antibody; Cardiomyopathy dilated 3A(X linked) antibody; EFE2 antibody; Endocardial fibroelastosis 2 antibody; Protein G4.5 antibody; Tafazzin antibody; TAZ antibody; TAZ protein antibody; TAZ_HUMAN antibody]

[Tafazzin; Tafazzin; tafazzin; protein G4.5; tafazzin; Protein G4.5]

[TAZ]

[TAZ; TAZ; EFE; BTHS; EFE2; G4.5; Taz1; CMD3A; LVNCX; EFE2; G4.5]

TAZ_HUMAN

Polyclonal

IgG

Rabbit

Reacts with: Human, Rat. Predicted to work with: Mouse

292

No cross reactivity with other species.

Immunogen affinity purified.

Lyophilized

At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time. Avoid repeated freezing and thawing.

Western Blot (WB)

Western Blot (WB): . Concentration: 0.1-0.5ug/ml. Tested Species: Hu, Rat. Predicted Species: Mouse. Wester Blot: The detection limit for TAZ is approximately 10ng/lane under reducing condtions. Tested Species: In-house tested species with positive results. Predicted Species: Species predicted to be fit for the product based on sequence similarities. Other applications have not been tested. Optimal dilutions should be determined by end users.

Western Blot (WB)

Immunogen: A synthetic peptide corresponding to a sequence in the middle region of human Tafazzin (162-178aa MDFILEKLNHGDWVHIF), identical to the related mouse and rat sequences.

Contents: Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3. Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Description: Rabbit IgG polyclonal antibody for Tafazzin(TAZ) detection. Tested with WB in Human, Mouse, Rat. Background: Tafazzin, also known as G4.5 is a protein that in humans is encoded by the TAZ gene. Cardiolipin is a complex glycerophospholipid with 4 acyl groups that localizes to the mitochondrial inner membrane and has a role in mitochondrial structure and function. By positional cloning, TAZ was identified within the critical Barth syndrome region on Xq28. Tafazzin is involved in the metabolism of cardiolipin. It is a component of the hippo signaling pathway thatcontrols tissue growth in animals. And it can function as aphospholipid lysophospholipid transacylase.

2498992

Q16635.1

Q16635

25,836 Da

Acyl Chain Remodeling Of CL Pathway (645330); Glycerophospholipid Biosynthesis Pathway (645313); Glycerophospholipid Metabolism Pathway (82989); Glycerophospholipid Metabolism Pathway (364); Metabolism Pathway (477135); Metabolism Of Lipids And Lipoproteins Pathway (160976); Metabolism Of Proteins Pathway (106230); Mitochondrial Protein Import Pathway (576261); Phospholipid Metabolism Pathway (645312)

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: mitochondrion; mitochondrial membrane; mitochondrial inner membrane; integral to membrane; intrinsic to membrane; cytosol; nucleus. Molecular Function: 1-acylglycerophosphocholine O-acyltransferase activity. Biological Process: cardiolipin biosynthetic process; cardiac muscle development; mitochondrial respiratory chain complex I assembly; skeletal muscle development; muscle contraction; cristae formation; phospholipid metabolic process; heart development; glycerophospholipid biosynthetic process; hemopoiesis; cardiac muscle contraction; organelle ATP synthesis coupled electron transport. Disease: Barth Syndrome

0.1 mg

280 USD