MTO1 recombinant protein

Recombinant Human MTO1

Cat. Num. AAA7613113

• Gene Names: MTO1; CGI-02; COXPD10
• Applications: Western Blot, ELISA
• Purity: Greater than 75% by SDS-PAGE gel analyses
• Synonyms: MTO1; Recombinant Human MTO1; CGI 02; MTO1 recombinant protein

• Host: E Coli
• Purity/Purification: Greater than 75% by SDS-PAGE gel analyses
• Form/Format: Lyophilized from a 0.2um filtered solution in PBS with 5% trehalose, pH7.4
• Sequence Positions: 549-717

• Applicable Applications for MTO1 recombinant protein: Western Blot (WB), ELISA (EIA)
• Species: Human
• Tag: N-terminal His-IF2DI Tag
• Reconstitution: Centrifuge the vial at 10,000rpm for 1 minute, reconstitute at 200ug/ml in sterile distilled water
• Preparation and Storage: Store at -20 degree C for 12 months as lyophilized; 2-8 degree C for 1 month under sterile conditions after reconstitution

NCBI and Uniprot Product Information

• NCBI GI #: 74024895
• NCBI GeneID: 25821
• NCBI Accession #: NP_036255.2
• NCBI GenBank Nucleotide #: NM_012123.3
• UniProt Accession #: Q9Y2Z2; Q5SWL2; Q5SWL3; Q5SWL4; Q8NDN7; Q8WZ57; Q96FE6; Q9BS06; B3KQB5
• Molecular Weight: 81,538 Da
• NCBI Official Full Name: protein MTO1 homolog, mitochondrial isoform a
• NCBI Official Synonym Full Names: mitochondrial tRNA translation optimization 1
• NCBI Official Symbol: MTO1
• NCBI Official Synonym Symbols: CGI-02; COXPD10
• NCBI Protein Information: protein MTO1 homolog, mitochondrial; mitochondrial MTO1-3; homolog of yeast Mto1; mitochondrial translation optimization 1 homolog
• UniProt Protein Name: Protein MTO1 homolog, mitochondrial
• Protein Family: Mitochondrial translation optimization protein
• UniProt Gene Name: MTO1
• UniProt Entry Name: MTO1_HUMAN

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