• Tafazzin Polyclonal Antibody
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YP-Ab-04235-100UL

Tafazzin Polyclonal Antibody

Tafazzin Polyclonal Antibody

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  • Gene Name: TAZ
  • Immunogen: Synthesized peptide derived from the Internal region of human Tafazzin.
  • Specificity: Tafazzin Polyclonal Antibody detects endogenous levels of Tafazzin protein.
  • Composition: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • Source: Polyclonal, Rabbit,IgG
  • Dilution ratio: Western Blot: 1/500 - 1/2000. ELISA: 1/5000. Not yet tested in other applications.
  • Purification process: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • Concentration: 1 mg/ml
  • Storage: -20°C/1 year
  • Other Names: TAZ; EFE2; G4.5; Tafazzin; Protein G4.5
  • Background: 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],
  • Function: disease:Defects in TAZ are the cause of 3-methylglutaconic aciduria type 2 (MGA2) [MIM:302060]. MGA2 is a severe metabolic disorder, often fatal in childhood, characterized by dilated cardiomyopathy, skeletal myopathy, short stature, neutropenia and 3-methylglutaconicaciduria.,disease:Defects in TAZ are the cause of non-compaction of left ventricular myocardium isolated X-linked (LVNCX) [MIM:300183]. LVNC is due to an arrest of myocardial morphogenesis. The disorder is characterized by a hypertrophic left ventricular with deep trabeculations and with poor systolic function, with or without associated left ventricular dilation. In some cases, the right ventricle is also affected.,domain:The hydrophilic domain may serve as an exposed loop interacting with other proteins.,function:Some isoforms may be involved in cardiolipin metabolism.,online information:TAZ mutation db,similarity:Belongs
  • Species: Human;Rat;Mouse;
  • Range: WB;ELISA
  • Protein: Tafazzin
  • DA: 33kD
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