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The CCA-adding enzyme TRNT1 (EC 22.214.171.124) is an essential enzyme that catalyzes the addition of the CCA terminus to the 3-prime end of tRNA precursors. Zusätzlich bieten wir Ihnen Trnt1 Antikörper (10) und Trnt1 Kits (1) und viele weitere Produktgruppen zu diesem Protein an.
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In vitro studies of disease-linked variants of human tRNA nucleotidyltransferase reveal decreased thermal stability and altered catalytic activity.
patient-specific induced pluripotent stem cells (iPSCs) and iPSC-derived retinal organoids from dermal fibroblasts of patients with molecularly confirmed TRNT1-associated retinitis pigmentosa.
Data show that the disease causing mutations in patient-derived fibroblasts do not affect subcellular localization of TRNT1 and show no gross morphological differences when compared to control cells.
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 (zeige FBN2 Proteine) addition to mitochondrial tRNA(Ser (zeige SIGLEC1 Proteine)(AGY)).
Tandem CCA (zeige FBN2 Proteine) 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 (zeige FBN2 Proteine)-adding enzyme on a substrate with increased conformational flexibility, the CCA (zeige FBN2 Proteine)-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 (zeige FBN2 Proteine)-adding enzyme and not a poly(A) polymerase (zeige PAPOLA Proteine).
These findings strongly suggest that the splice variant of the human CCA (zeige FBN2 Proteine)-adding enzyme is expressed in the cell although the in vivo function remains unclear.
The CCA-adding enzyme TRNT1 (EC 126.96.36.199) 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