TDP-43
Pronunciation: TEE-dee-PEE-forty-three
Also written as: TDP-43 — TAR DNA-binding protein 43
TAR DNA-binding protein 43, a pathological protein aggregate found in frontotemporal dementia and ALS.
Full Definition
TDP-43 (TAR DNA-binding protein 43) is a nuclear RNA-binding protein that forms pathological cytoplasmic inclusions in several neurodegenerative diseases, most notably frontotemporal lobar degeneration (FTLD-TDP) and amyotrophic lateral sclerosis (ALS). In disease states, TDP-43 becomes hyperphosphorylated, ubiquitinated, and cleaved, forming characteristic cytoplasmic inclusions while being depleted from the nucleus. These inclusions can be detected using specific antibodies against phosphorylated TDP-43 and appear as various morphologies including skein-like inclusions, dot-like inclusions, and dystrophic neurites. TDP-43 pathology has become a defining feature of FTLD-TDP and has been found in other conditions including Alzheimer's disease and chronic traumatic encephalopathy.
Usage
Usage note: Key biomarker for FTLD-TDP; specify inclusion morphology and distribution.
In Context
- "Phosphorylated TDP-43 immunostaining revealed numerous cytoplasmic inclusions in the frontal cortex." — FTLD diagnosis
- "The motor neurons showed characteristic TDP-43 skein-like inclusions consistent with ALS pathology." — Motor neuron disease case