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Products:Signal Transduction >> Metabolism >> Lipid metabolism
Overview
Product name
Tafazzin / TAZ peptide (276-287)
See all Tafazzin / TAZ products (2) ...
Protein description
Synthetic peptide: C-HLKTQAEQLHNH, corresponding to C terminal amino acids 276-287 of Human Tafazzin/TAZ. This peptide was used as an immunogen for ab40004 - Tafazzin / TAZ antibody.
Properties
Form
Liquid
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Concentration
Concentration information loading...
Sequence
C-HLKTQAEQ LHNH
Research areas
Protein info
Function
Some isoforms may be involved in cardiolipin (CL) metabolism.
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.
Involvement in disease
Defects in TAZ are the cause of 3-methylglutaconic aciduria type 2 (MGA2) [MIM:302060]; also known as Barth syndrome. MGA2 is a severe metabolic disorder, often fatal in childhood, characterized by dilated cardiomyopathy, skeletal myopathy, short stature, neutropenia and 3-methylglutaconicaciduria.
Defects in TAZ are the cause of left ventricular non-compaction 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.
Sequence similarities
Belongs to the taffazin family.
Domain
The hydrophilic domain may serve as an exposed loop interacting with other proteins.
Cellular localization
Cytoplasm and Membrane.
Target information above from: UniProt accessionQ16635
The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010).
References for Tafazzin / TAZ peptide (276-287) (ab45823)
ab45823 has not yet been referenced specifically in any publications.
Publishing research using ab45823? Please let us know so that we can cite the reference in this datasheet
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"