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The antibody against NSUN3 was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 1-120 of human NSUN3 (NP_071355.1) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, ELISA.
The antibody against NSUN3 was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 1-120 of human NSUN3 (NP_071355.1) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, ELISA.
| Cat.No | ADA-09557A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | NSUN3 |
| Target Synonyms | MST077; COXPD48; MSTP077; NSUN3 | Form | Liquid |
| Species Reactivity | Human, Mouse, Rat | Isotype | IgG |
| Storage Buffer | 50% Glycerol, PBS with 0.01% thimerosal, pH7.3. | Purification Method | Affinity purification |
| Positive Samples | K-562, Mouse thymus, Rat thymus | Application | ELISA, WB |
| Immunogen Description | Recombinant fusion protein containing a sequence corresponding to amino acids 1-120 of human NSUN3 (NP_071355.1). | Target Species | Human |
|---|---|---|---|
| Immunogen Sequence | MLTQLKAKSEGKLAKQICKVVLDHFEKQYSKELGDAWNTVREILTSPSCWQYAVLLNRFNYPFELEKDLHLKGYHTLSQGSLPNYPKSVKCYLSRTPGRIPSERHQIGNLKKYYLLNAAS | Uniprot ID | Q9H649 |
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.
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