Alpha-Internexin antibody

Background of Alpha-Internexin antibody

In mammalian CNS, five key neurol intermediate filament (NIF) proteins have been discovered which includes alpha-internexin (66 kD, I or Inexa), peripherin (57 kD), and three neurofilament (NF) proteins, which are neurofilament light (NF-L, 68 kD), medium (NF-M, 145 kD), and heavy (NF-H, 200 kD). Among NIFs, alpha-internexin is categorized as class IV NIF which is highly expressed in most neurons during development especially when they begin to differentiate and before the expression of NF triplet proteins, and it is structurally/functiolly associated with NF triplet proteins inside mature CNS neurons. Peripherin is mainly expressed in PNS and in some CNS neurons also, and alpha-internexin - peripherin self-assemble or co-assemble with neurofilament protein subunits to form filamentous structure before their translocation into the axons to become an essential constituent of shape-maintaining IF network in mature neurons. It has been recognized as one of the many proteins phosphorylated by ATM/ATR in response to D damage and has been associated with developmental, degenerative /overexpression causes neurol cell death, and inflammatory processes of CNS. Abnormal NIF accumulation is a neuropathological sigture of several neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, dementia with Lewy bodies, and amyotrophic lateral sclerosis. Alpha-internexin has been detected as a major component of pathological inclusions in frontotemporal dementia, also called 'neurol intermediate filament inclusion disease (NIFID).