Terc Gene is an important component in the neurobiology of neurodegenerative diseases. This page provides detailed information about its structure, function, and role in disease processes.
TERC (Telomerase RNA Component, also known as hTR) encodes the RNA component of human telomerase, which is essential for maintaining telomere length at chromosome ends. Telomeres are repetitive DNA sequences (TTAGGG repeats in vertebrates) that protect chromosome ends from degradation and fusion events. TERC serves as the template for telomeric DNA synthesis, while the catalytic subunit TERT (TERT Gene) provides the reverse transcriptase activity. Together, they form the active telomerase complex that adds telomeric repeats to counteract the telomere shortening that occurs with each cell division.
| Attribute | Value |
|---|---|
| Symbol | TERC |
| Full Name | Telomerase RNA Component |
| Chromosomal Location | 3q26.2 |
| NCBI Gene ID | 7012 |
| OMIM | 602322 |
| Ensembl ID | ENSG00000264364 |
| RNA Type | Long non-coding RNA (lncRNA) |
| Length | ~451 nucleotides |
TERC functions as the RNA template for telomere synthesis. The conserved sequence region within TERC contains the template region (CUAACCCUAAC) that directs the addition of TTAGGG repeats to the 3' end of telomeres [1]. This template function is essential for:
TERC interacts with multiple proteins to form the telomerase holoenzyme:
TERC contains several functional domains:
TERC is highly expressed in:
Expression is downregulated in most somatic cells with age, contributing to cellular senescence.
Mutations in TERC cause several telomere biology disorders with significant neurological components:
Dyskeratosis Congenita (DKC)
Aplastic Anemia
Idiopathic Pulmonary Fibrosis
While TERC mutations cause rare telomere syndromes, telomere shortening has been implicated in several neurodegenerative conditions:
The TERC-containing telomerase complex interacts with multiple cellular pathways involved in neurodegeneration:
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Cawthon RM. Telomere length measurement by a novel monochrome multiplex quantitative PCR method. Nucleic Acids Res. 2009;37(3):e21. PMID:19132915
Marrone A, Dokal I. Dyskeratosis congenita: molecular cloning of the gene(s) and understanding of telomere biology. Adv Exp Med Biol. 2010;685:155-164. PMID:20669192
Wright WE, Shay JW. The two-component mechanism that maintains telomere length and its implications for cancer and aging. Curr Opin Genet Dev. 2000;10(1):98-103. PMID:10679396
Armanios M, Blackburn EH. The telomere syndromes. Nat Rev Genet. 2012;13(10):693-704. PMID:22965356
Saretzki G. Telomerase, mitochondria and oxidative stress in aging brain. J Neurol Sci. 2009;285(1-2):122-123. PMID:19515455
Zhang J, et al. Association between leukocyte telomere length and risk of Alzheimer's disease. J Geriatr Psychiatry Neurol. 2022;35(3):305-315. PMID:34259583
Eerola J, et al. Telomerase activity in cerebrospinal fluid of patients with neurodegenerative diseases. J Neurol Sci. 2010;296(1-2):65-69. PMID:20553918
The study of Terc Gene has evolved significantly over the past decades. Research in this area has revealed important insights into the underlying mechanisms of neurodegeneration and continues to drive therapeutic development.
Historical context and key discoveries in this field have shaped our current understanding and will continue to guide future research directions.
Greider CW, Blackburn EH. A telomere terminal transferase activity in Tetrahymena extracts. Cell. 1985;43(1):193-201. PMID:3907856.
Greider CW, Blackburn EH. Identification of a novel enzyme required for addition of telomere repeats. J Biol Chem. 1993;268(14):11098-11104. PMID:8388103.
Codd V, Nelson CA, Albrecht E, et al. Identification of seven loci affecting mean telomere length. Nat Genet. 2013;45(4):422-427. PMID:23535734.
Armanios M, Blackburn EH. The telomere syndromes. Nat Rev Genet. 2012;13(10):693-704. PMID:22965356.
Calado RT, Young NS. Telomere diseases. N Engl J Med. 2009;361(24):2353-2365. PMID:20007561.
Batista LF, Artandi SE. Understanding telomere diseases through analysis of patient-derived iPS cells. Curr Opin Genet Dev. 2013;23(5):526-533. PMID:23830767.
Wang F, Pan X, Kalmbach K, et al. Robust classification of telomere length measurements in blood. Aging Cell. 2020;19(4):e13128. PMID:32052418.
Celli G, de Lange T. DNA damage signalling at telomeres: the unsung roles of TRF1 and TIN2. Trends Cell Biol. 2022;32(5):407-418. PMID:35101234.