TRNT1 polymerizes CCA at the 3' end of pre-tRNA

Stable Identifier
R-HSA-6786881
Type
Reaction [transition]
Species
Homo sapiens
Compartment
mitochondrial matrix
ReviewStatus
5/5
Locations in the PathwayBrowser
Expand all
General
SVG | 
PNG
Low
Medium
High
 | PPTX  | SBGN
TRNT1 polymerizes CCA at the 3' end of pre-tRNA
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout
TRNT1 (CCA-adding enzyme) polymerizes a nontemplated CCA sequence on the 3' end of mitochondrial tRNA (Nagaike et al. 2001). Mutations in TRNT1 or tRNAs that affect the rate of CCA addition cause pathological consequences in humans (Tomari et al. 2003, Chakraborty et al. 2014, Sasarman et al. 2015).
Literature References
PubMed ID Title Journal Year
12621050 Decreased CCA-addition in human mitochondrial tRNAs bearing a pathogenic A4317G or A10044G mutation

Suzuki, T, Nagaike, T, Hino, N, Ueda, T, Tomari, Y

J. Biol. Chem. 2003
11504732 Identification and characterization of mammalian mitochondrial tRNA nucleotidyltransferases

Takemoto-Hori, C, Nagaike, T, Watanabe, K, Ueda, T, Negayama, F, Tomari, Y, Suzuki, T

J. Biol. Chem. 2001
25193871 Mutations in TRNT1 cause congenital sideroblastic anemia with immunodeficiency, fevers, and developmental delay (SIFD)

Naas, T, Lau, A, Durie, D, Moppett, J, Campagna, DR, Giardina, PJ, Geraghty, M, Fleming, MD, Jolles, S, Schmitz-Abe, K, Thompson, AA, Joyce, PB, Kennedy, EK, Wiseman, DH, Holcik, M, Bonney, DK, Chakraborty, PK, Kannengiesser, C, Hughes, S, Mamady, H, Minniti, CP, Wynn, RF, Marques, L, Heeney, MM, Sendamarai, AK, Connor, P, Markianos, K, Bordon, V, May, A, Laxer, RM, Bottomley, SS, Rudner, AD, Klaassen, RJ, Powell, C, Thuret, I

Blood 2014
25652405 The 3' addition of CCA to mitochondrial tRNASer(AGY) is specifically impaired in patients with mutations in the tRNA nucleotidyl transferase TRNT1

Elpeleg, O, Gauthier, J, Maftei, C, Shoubridge, EA, Weraarpachai, W, Brunel-Guitton, C, Antonicka, H, Ellazam, B, Mitchell, G, Webb, N, Salomon, S, Sasarman, F, Thiffault, I, Janer, A

Hum. Mol. Genet. 2015
Participants
Input
Output
Participates
as an event of
Event Information
Go Biological Process
mitochondrial tRNA 3'-end processing (1990180)
Catalyst Activity

5'-3' RNA polymerase activity of TRNT1 [mitochondrial matrix]

Physical Entity
TRNT1 [mitochondrial matrix] (Homo sapiens)
Activity
5'-3' RNA polymerase activity (GO:0034062)
Authored
May, B  (2015-07-09)
Reviewed
Suzuki, T  (2015-11-18)
Powell, CA  (2015-11-07)
Levinger, L  (2015-08-11)
Motorin, Y  (2015-08-25)
Jarrous, N  (2015-10-24)
Created
May, B  (2015-07-10)
Cite Us!