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Anti-Tafazzin/TAZ Antibody Picoband® Fluoro647 Conjugated

Product Specifications

Background

Tafazzin?is a?protein?that in humans is encoded by the?TAFAZZIN?gene. 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.

Synonyms

Tafazzin; Protein G4.5; TAZ; EFE2, G4.5

Gene Name

TAZ

Gene ID

6901

UniProt

Q16635

Host

Rabbit

Reactivity

Mouse, Rat

Cross Reactivity

No cross-reactivity with other proteins

Immunogen

A synthetic peptide corresponding to a sequence in the middle region of human Tafazzin, identical to the related mouse and rat sequences.

Clonality

Polyclonal

Tissue Specificity

High levels in cardiac and skeletal muscle. Up to 10 isoforms can be present in different amounts in different tissues. Most isoforms are ubiquitous. Isoforms that lack the N- terminus are found in leukocytes and fibroblasts, but not in heart and skeletal muscle. Some forms appear restricted to cardiac and skeletal muscle or to leukocytes.

Applications

Flow Cytometry

Field of Research

Stem Cells, Transcription

Purification

Immunogen affinity purified.

Form

Liquid

Function

Some isoforms may be involved in cardiolipin (CL) metabolism. .

References & Citations

1. Acehan, D., Vaz, F., Houtkooper, R. H., James, J., Moore, V., Tokunaga, C., Kulik, W., Wansapura, J., Toth, M. J., Strauss, A., Khuchua, Z. Cardiac and skeletal muscle defects in a mouse model of human Barth syndrome. J. Biol. Chem. 286: 899-908, 2011. 2. Barth, P. G., Valianpour, F., Bowen, V. M., Lam, J., Duran, M., Vaz, F. M., Wanders, R. J. A. X-linked cardioskeletal myopathy and neutropenia (Barth syndrome) : an update. Am. J. Med. Genet. 126A: 349-354, 2004. 3. Claypool, S. M., McCaffery, J. M., Koehler, C. M. Mitochondrial mislocalization and altered assembly of a cluster of Barth syndrome mutant tafazzins. J. Cell Biol. 174: 379-390, 2006. 4. Hastings, R., Steward, C., Tsai-Goodman, B., Newbury-Ecob, R. Dysmorphology of Barth syndrome. Clin. Dysmorph. 18: 185-187, 2009.

Storage Conditions

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

Calculated Molecular Weight

33 kDa

Specificity

No cross reactivity with other proteins.

Applications Notes

6

Gene Name Synonym

Tafazzin

Subcellular Location

Isoform 1: Membrane; Single-pass membrane protein.

Sequence Similarities

Belongs to the taffazin family.

Protein Name

Tafazzin

Isotype

Rabbit IgG

Contents

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.

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