Specificity
This antibody recognises bovine intermediate 200 kDa Neurofilament H (NFH), a 1026 amino acid ~200 kDa protein involved in the maintenance of neuronal integrity.Neurofilaments are a major component of the cellular cytoskeleton, acting as the most abundant support for the axon cytoplasm. They exist as three neurofilament proteins: 68/70 kDa (NFL) 160 kDa (NFM) and 200 kDa (NFH), which are expressed in both the central and peripheral nervous systems. Although typically expressed by neurons, neurofilaments are also expressed by neuroblastomas, neuromas, gangliogliomas, ganglioneuromas, and ganglioneuroblastomas, as well as paragangliomas, carcinoids, skin neuroendocrine carcinomas, and oat cell carcinomas of the lung. Defects in NFH are responsible for susceptibility to the neurodegenerative disorder amyotrophic lateral sclerosis (ALS) which affects upper and lower motor neurons, and results in fatal paralysis (Mendonça et al. 2005).