Human Tafazzin (TAZ) ELISA Kit

The Tafazzin (TAZ) protein is a phospholipid-lysophospholipid transacylase localized to the inner mitochondrial membrane, where it remodels nascent cardiolipin into the mature tetralinoleoyl-cardiolipin species that stabilize respiratory chain supercomplexes and cristae architecture. Loss-of-function mutations in the X-linked TAZ gene cause Barth syndrome, an inherited mitochondrial disorder characterized in affected males by dilated cardiomyopathy, left ventricular noncompaction, neutropenia, skeletal myopathy, and growth delay, while carrier females may show subclinical cardiac phenotypes. Quantitative measurement of TAZ protein therefore supports translational research into cardiolipin metabolism, mitochondrial bioenergetics, and the pathogenesis of Barth syndrome.

This Assay Genie kit is supplied as a complete microplate-format package intended for research use only, with capture antibody precoated wells and all buffers, standards, and detection reagents provided in individually labeled vials. Researchers load a 100 uL sample volume per well, and standard reagents should be stored frozen at -20 °C prior to first use; repeated freeze-thaw cycles of the standard should be avoided by aliquoting upon receipt. The assay can be applied to cultured cardiomyocyte lysates, patient-derived fibroblasts, induced pluripotent stem cell-derived cardiac models, and tissue homogenates from rodent models of mitochondrial dysfunction to monitor TAZ expression under pharmacological or genetic perturbation.