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The CCA-adding enzyme TRNT1 (EC 184.108.40.206) is an essential enzyme that catalyzes the addition of the CCA terminus to the 3-prime end of tRNA precursors. Additionally we are shipping Trnt1 Antibodies (11) and and many more products for this protein.
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family expands the ocular and systemic phenotypes associated with mutations in TRNT1, demonstrating phenotypic variability and highlighting the need for ophthalmic review of these patients.
two non-syndromic retinitis pigmentosa pedigrees with segregating mutations in TRNT1
The clinical phenotypes associated with TRNT1 mutations are largely due to impaired mitochondrial translation, resulting from defective CCA (show FBN2 Proteins) addition to mitochondrial tRNA(Ser (show SIGLEC1 Proteins)(AGY)).
Tandem CCA (show FBN2 Proteins) addition is not the result of a modified enzymatic activity that is particular to unstable RNAs. Rather, it is a consequence of the natural activity of the CCA (show FBN2 Proteins)-adding enzyme on a substrate with increased conformational flexibility, the CCA (show FBN2 Proteins)-adding enzyme is able to trigger the degradation of potentially detrimental small RNAs and tRNAs.
The patient-associated TRNT1 mutations result in partial loss of function of TRNT1 and lead to metabolic defects in both the mitochondria and cytosol, which can account for the phenotypic pleiotropy.
human gene transcript CGI-47 (#AF151805) was cloned and encodes a bona fide CCA (show FBN2 Proteins)-adding enzyme and not a poly(A) polymerase (show PAPOLA Proteins).
These findings strongly suggest that the splice variant of the human CCA (show FBN2 Proteins)-adding enzyme is expressed in the cell although the in vivo function remains unclear.
is a RNA polymerase which newly adds CCA (show FBN2 Proteins) sequence to tRNA 3'terminal. This reaction was named as Vice-Anchored Knock-in and Lock Dynamics.[review]
The CCA-adding enzyme TRNT1 (EC 220.127.116.11) is an essential enzyme that catalyzes the addition of the CCA terminus to the 3-prime end of tRNA precursors. This reaction is a fundamental prerequisite for mature tRNAs to become aminoacylated and to participate in protein biosynthesis (Lizano et al., 2007
CCA tRNA nucleotidyltransferase 1, mitochondrial
, tRNA-nucleotidyltransferase 1, mitochondrial
, tRNA nucleotidyl transferase, CCA-adding, 1
, tRNA-nucleotidyltransferase 1, mitochondrial-like
, mitochondrial CCA-adding tRNA-nucleotidyltransferase
, mitochondrial tRNA nucleotidyl transferase, CCA-adding
, mt CCA-adding enzyme
, mt tRNA CCA-diphosphorylase
, mt tRNA CCA-pyrophosphorylase
, mt tRNA adenylyltransferase
, tRNA adenylyltransferase, mitochondrial