PERIPHERIN Meaning and
Definition
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Peripherin is a type III intermediate filament protein that is primarily found in the peripheral nervous system and is encoded by the PRPH gene. It is classified as a cytoskeletal protein due to its role in providing structural support and stability to cells. Peripherin is specifically expressed in neurons of the myelinated and unmyelinated sensory and motor nerves, as well as in certain neuroendocrine cells.
In terms of its structure, peripherin consists of a central α-helical rod domain flanked by non-helical N- and C-terminal domains. The rod domain enables peripherin to form filaments, which are long, fibrous structures involved in maintaining cellular integrity and regulating various cellular processes. These filaments help to provide structural support along the axons, dendrites, and cell bodies of neurons.
Functionally, peripherin plays a crucial role in axonal growth, regeneration, and maintenance. It is involved in the organization and bundling of neurofilaments, which are essential components of the neuronal cytoskeleton. Additionally, peripherin regulates axonal transport and influences the localization of various proteins within neurons.
Mutations in the PRPH gene can lead to the development of peripheral neuropathies, including Charcot-Marie-Tooth disease type 2E (CMT2E) and amyotrophic lateral sclerosis (ALS). These conditions are characterized by degeneration and dysfunction of the peripheral nerves, resulting in symptoms such as muscle weakness, loss of sensation, and impaired motor coordination.
In summary, peripherin is a cytoskeletal protein abundantly found in the peripheral nervous system. It is essential for maintaining the structure and function of neurons, and its abnormalities can contribute to various peripheral neuropathies.
Etymology of PERIPHERIN
The word "peripherin" is derived from the combination of two words: "peripheral" and "in" as a suffix.
- "Peripheral" is derived from the Latin word "peripheria", which means "outer area" or "circumference". It entered English in the late 16th century and initially referred to the outer parts of a region or system.
- "In" is a common suffix in English that is derived from the Old English word "innan", meaning "inside" or "within". It is often used to form adjectives indicating being located or found within something.
Therefore, "peripherin" combines these two elements to describe something located within or associated with the periphery or outer areas of something. In specific contexts, "peripherin" is commonly used to refer to a specific protein called "peripherin" that is found in the peripheral nervous system.
