Anti-Human Neurofilament Heavy Polypeptide Antibody Pair

Product Description: Neurofilament heavy chain (NfH) is one of the subunits that make up neurofilaments, which are major cytoskeletal components of neurons. NfH forms the side arms of the neurofilament structure, with neurofilament light chain (NfL) forming the backbone. Abnormal accumulation or altered phosphorylation of NfH is implicated in various neurodegenerative diseases. In Alzheimer's disease, NfH is hyperphosphorylated and an integral component of neurofibrillary tangles. In Parkinson's disease, NfH is found in Lewy bodies and forms a cage-like structure around them. NfH accumulates excessively in axons in giant axonal neuropathy. In amyotrophic lateral sclerosis (ALS), NfH levels in cerebrospinal fluid and blood are highly elevated, correlating with disease progression and survival times.Autoantibodies against NfH aggregates may contribute to the variable expression of plasma NfH levels during ALS progression.,The neurofilament heavy polypeptide (NEFH) is a crucial component of neurofilaments, which are intermediate filaments providing structural support in neurons. NEFH plays a significant role in maintaining the integrity of podocytes, specialized cells in the kidney, by preventing injury and detachment, particularly in conditions like focal segmental glomerulosclerosis and membranous nephropathy. In the nervous system, NEFH contributes to the structural support of axons and is involved in the radial expansion necessary for nerve conduction. Mutations in the NEFH gene can lead to neurodegenerative diseases such as Charcot-Marie-Tooth disease type 2CC, characterized by amyloidogenic protein aggregation causing muscle weakness and atrophy. Additionally, NEFH mutations are implicated in amyotrophic lateral sclerosis (ALS), where they disrupt axonal transport, leading to neurodegeneration. These insights highlight the dual role of NEFH in both structural maintenance and disease pathology across different tissues. Protein Function: Neurofilaments usually contain three intermediate filament proteins: NEFL, NEFM, and NEFH which are involved in the maintenance of neuronal caliber. NEFH has an important function in mature axons that is not subserved by the two smaller NF proteins. May additionally cooperate with the neuronal intermediate filament proteins PRPH and INA to form neuronal filamentous networks (By similarity) [The Uniprot Consortium]