MTO1 Antibody (PACO10651)
- SKU:
- PACO10651
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
抗体名: | MTO1 Antibody (PACO10651) |
抗体コード: | PACO10651 |
サイズ: | 50ul |
宿主種: | Rabbit |
申し込み: | ELISA, WB |
推奨される希釈: | |
反応性: | Human, Mouse, Rat |
免疫原: | Human MTO1 |
憲法: | Liquid |
ストレージバッファ: | PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles. |
精製方法: | Antigen Affinity purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Non-conjugated |
シノニム: | mitochondrial translation optimization 1 homolog (S. cerevisiae);MTO1;CGI-02 ; |
UniProt Protein Function: | MTO1: Involved in the 5-carboxymethylaminomethyl modification (mnm(5)s(2)U34) of the wobble uridine base in mitochondrial tRNAs. Defects in MTO1 are the cause of combined oxidative phosphorylation deficiency type 10 (COXPD10). An autosomal recessive disorder resulting in variable defects of mitochondrial oxidative respiration. Affected individuals present in infancy with hypertrophic cardiomyopathy and lactic acidosis. The severity is variable, but can be fatal in the most severe cases. Belongs to the MnmG family. 6 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:RNA processing Chromosomal Location of Human Ortholog: 6q13 Cellular Component: mitochondrion Molecular Function:FAD binding Disease: Combined Oxidative Phosphorylation Deficiency 10 |
NCBI Summary: | This gene encodes a mitochondrial protein thought to be involved in mitochondrial tRNA modification. The encoded protein may also play a role in the expression of the non-syndromic and aminoglycoside-induced deafness phenotypes associated with a specific mutation in the mitochondrial 12S rRNA gene. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008] |
UniProt Code: | Q9Y2Z2 |
NCBI GenInfo Identifier: | 183227703 |
NCBI Gene ID: | 25821 |
NCBI Accession: | NP_001116698.1 |
UniProt Secondary Accession: | Q9Y2Z2,Q5SWL2, Q5SWL3, Q5SWL4, Q8NDN7, Q8WZ57, Q96FE6 Q9BS06, B3KQB5, |
Molecular Weight: | 81,538 Da |
NCBI Full Name: | protein MTO1 homolog, mitochondrial isoform c |
UniProt Protein Name: | Protein MTO1 homolog, mitochondrial |
Protein Family: | Mitochondrial translation optimization protein |
UniProt Gene Name: | MTO1 |
UniProt Entry Name: | MTO1_HUMAN |