Rabbit MTO1 Polyclonal Antibody | anti-MTO1 antibody
MTO1 Polyclonal Antibody
NCBI and Uniprot Product Information
NCBI Description
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 Description
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.
Protein type: RNA processing
Chromosomal Location of Human Ortholog: 6q13
Cellular Component: mitochondrion
Molecular Function: FAD binding
Disease: Combined Oxidative Phosphorylation Deficiency 10
Research Articles on MTO1
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Product Notes
The MTO1 mto1 (Catalog #AAA8574163) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The MTO1 Polyclonal Antibody reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's MTO1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). WB: 1:500-1:2000. Researchers should empirically determine the suitability of the MTO1 mto1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "MTO1, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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