NSUN3 Antibody, FITC conjugated

Datasheet

Code	CSB-PA867156LC01HU
Size	US$166
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Product Details
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Target Background

Product Details

Full Product Name

Rabbit anti-Homo sapiens (Human) NSUN3 Polyclonal antibody

Uniprot No.

Q9H649

Target Names

NSUN3

Alternative Names

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

Raised in

Rabbit

Species Reactivity

Human

Immunogen

Recombinant Human tRNA (cytosine(34)-C(5))-methyltransferase, mitochondrial protein (1-200AA)

Immunogen Species

Homo sapiens (Human)

Conjugate

FITC

Clonality

Polyclonal

Isotype

IgG

Purification Method

>95%, Protein G purified

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4

Form

Liquid

Troubleshooting and FAQs

Antibody FAQs

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Lead Time

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Usage

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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Target Background

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.

Gene References into Functions

The authors show that the RNA methyltransferase NSUN3 localises to mitochondria and interacts with mt-tRNA(M)(et) to methylate cytosine 34 (C34) at the wobble position. PMID: 27497299
The NSUN3-knockout cells showed strong reduction in mitochondrial protein synthesis and reduced oxygen consumption, leading to deficient mitochondrial activity. It reconstituted formation of 5-methylcytidine (m(5)C) at position 34 (m(5)C34) on mt-tRNA(Met) with recombinant NSUN3 in the presence of AdoMet, demonstrating that NSUN3-mediated m(5)C34 formation initiates f(5)C34 biogenesis. PMID: 27214402

Involvement in disease

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.

Subcellular Location

Mitochondrion matrix.

Protein Families

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

Database Links

HGNC: 26208

OMIM: 617491

KEGG: hsa:63899

STRING: 9606.ENSP00000318986

UniGene: Hs.436594

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