Recombinant Human Putative methyltransferase NSUN3 (NSUN3)

Cat.No	ACP11821	Target Name	NSUN3
Form	Lyophilized powder	Expression System	Custom Production. Please inquire and provide the desire expression system.
Expression Range	1-340	Protein Length	Full length protein
Purity	>85% (SDS-PAGE)	Storage Buffer	5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0.

Cat.No

ACP11821

Target Name

NSUN3

Form

Lyophilized powder

Expression System

Custom Production. Please inquire and provide the desire expression system.

Expression Range

1-340

Protein Length

Full length protein

Purity

>85% (SDS-PAGE)

Storage Buffer

5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0.

Target Species	Human	Uniprot ID	Q9H649

Background Information

Uniprot Id

Q9H649
Target Species

Human
Target Name

NSUN3
Target Full Name

tRNA (cytosine(34)-C(5))-methyltransferase, mitochondrial
Target Function

Mitochondrial tRNA methyltransferase that mediates methylation of cytosine to 5-methylcytosine (m5C) at position 34 of mt-tRNA(Met). mt-tRNA(Met) methylation at cytosine(34) takes place at the wobble position of the anticodon and initiates the formation of 5-formylcytosine (f(5)c) at this position. mt-tRNA(Met) containing the f(5)c modification at the wobble position enables recognition of the AUA codon in addition to the AUG codon, expanding codon recognition in mitochondrial translation.
Target Involvement

Mitochondrial complex deficiency (PubMed:27356879). A disorder of the mitochondrial respiratory chain resulting in a highly variable phenotype depending on which tissues are affected (PubMed:27356879). Clinical features include combined developmental disability, microcephaly, failure to thrive, recurrent increased lactate levels in plasma, muscular weakness, proximal accentuated, external ophthalmoplegia and convergence nystagmus (PubMed:27356879). Defects are probably caused by deficient methylation and formylation of mt-tRNA(Met) wobble cytosine (PubMed:27356879). The disease may be caused by mutations affecting the gene represented in this entry.
Target Subcellular Location

Mitochondrion matrix.
Target Protein Families

Class I-like SAM-binding methyltransferase superfamily, RsmB/NOP family
Target Synonyms

6720484A09Rik; AU022521; FLJ22109; FLJ22609; MST077; MSTP077; NOL1/NOP2/Sun domain family 3; NOL1/NOP2/Sun domain family member 3; NOP2/Sun domain family member 3; NSUN3; NSUN3_HUMAN; OTTMUSP00000028633; Putative methyltransferase NSUN3; UG0651E06
Target Background

Enables tRNA (cytosine-5-)-methyltransferase activity. Involved in regulation of mitochondrial translation and tRNA wobble base cytosine methylation. Located in mitochondrial matrix. Implicated in combined oxidative phosphorylation deficiency 48.

Recombinant Human Putative methyltransferase NSUN3 (NSUN3)

Specifications

Immunogen Information

Inquire Recombinant Human Putative methyltransferase NSUN3 (NSUN3) Now

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