MTO1 Antibody (PACO45608)
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주문- SKU:
- PACO45608
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | MTO1 Antibody (PACO45608) |
Antibody SKU: | PACO45608 |
Size: | 50ul |
Host Species: | Rabbit |
Tested Applications: | ELISA, WB, IHC |
Recommended Dilutions: | ELISA:1:2000-1:10000, WB:1:1000-1:5000, IHC:1:20-1:200 |
Species Reactivity: | Human, Rat |
Immunogen: | Recombinant Human Protein MTO1 homolog, mitochondrial protein (420-680AA) |
Form: | Liquid |
Storage Buffer: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Purification Method: | Antigen Affinity Purified |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
![]() | Western blot. All lanes: MTO1 antibody at 2.25µg/ml. Lane 1: Rat heart tissue. Lane 2: Jurkat whole cell lysate. Secondary. Goat polyclonal to rabbit IgG at 1/10000 dilution. Predicted band size: 80, 67, 35, 78, 73, 82 kDa. Observed band size: 80, 67, 35 kDa.. |
![]() | Immunohistochemistry of paraffin-embedded human kidney tissue using PACO45608 at dilution of 1:100. |
![]() | Immunohistochemistry of paraffin-embedded human brain tissue using PACO45608 at dilution of 1:100. |
Background: | Involved in the 5-carboxymethylaminomethyl modification (mnm(5)s(2)U34) of the wobble uridine base in mitochondrial tRNAs. |
Synonyms: | Protein MTO1 homolog, mitochondrial, MTO1 |
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 |