Titin is a nucleolar protein in neurons.
Res Sq
; 2024 Mar 04.
Article
in En
| MEDLINE
| ID: mdl-38496572
ABSTRACT
Titin is the largest protein produced by living cells and its function as a molecular spring in striated muscle is well characterized (1, 2). Here we demonstrate that titin isoforms in the same size range as found in muscle are prominent neuronal proteins in both the central and peripheral nervous systems, including motor neurons in the spinal cord and brain. Within these neurons, titin localizes to the dense fibrillar component of the nucleolus, the site of ribosomal RNA biogenesis and modification, and a critical site of dysfunction in neurodegenerative disease (3-5). Additionally, we show that the levels of both titin mRNA and protein are altered in the spinal cord of SOD1G93A mice, a commonly used model of amyotrophic lateral sclerosis, indicating that titin mediated nucleolar events may in fact contribute to the pathobiology of disease.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Language:
En
Journal:
Res Sq
Year:
2024
Document type:
Article
Country of publication:
Estados Unidos