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

Polyclonal

IgG

Rabbit

The antibody is a rabbit polyclonal antibody raised against TAZ. It has been selected for its ability to recognize TAZ in immunohistochemical staining andwestern blotting.

Affinity Chromatography

Supplied as solution form in PBS, pH7.4, containing 0.02% NaN3,50% glycerol.

200ug/ml (lot specific)

Store at 4 degree C for frequent use. Stored at -20 degree C to -80 degree C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.

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 anti-TAZ antibody 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.

Immunocytochemistry (ICC), Immunohistochemistry (IHC) - Formalin/Paraffin, ELISA (EIA), Western Blot (WB)

Western blotting: 1:100-400
Immunocytochemistry in formalin fixed cells: 1:100-500
Immunohistochemistry in formalin fixed frozen section: 1:100-500
Immunohistochemistry in paraffin section: 1:50-200
Enzyme-linked Immunosorbent Assay: 1:100-200

Western Blot: Sample: Mouse Heart lysate; Primary Ab: 5ug/ml Rabbit Anti-Mouse TAZ Antibody Second Ab: 0.2ug/mL?HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody

Western Blot: Sample: Recombinant protein.

DAB staining on IHC-P. Samples: Mouse Tissue)

30,433 Da

tafazzin

tafazzin

Tafazzin

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

Chromosomal Location of Human Ortholog: X A7.3|X 37.95 cM

Cellular Component: cytoplasm; cytosol; intrinsic component of membrane; membrane; mitochondrial membrane; mitochondrion; nucleus

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

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

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