MTO1 Antibody

Code CSB-PA015191GA01HU
Size $600
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Product Details

Uniprot No.
Target Names
Alternative Names
MTO1 antibody; CGI-02 antibody; Protein MTO1 homolog antibody; mitochondrial antibody
Raised in
Species Reactivity
Human MTO1
Immunogen Species
Homo sapiens (Human)
Purification Method
Antigen Affinity purified
It differs from different batches. Please contact us to confirm it.
PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
Tested Applications
Troubleshooting and FAQs
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Involved in the 5-carboxymethylaminomethyl modification (mnm(5)s(2)U34) of the wobble uridine base in mitochondrial tRNAs.
Gene References into Functions
  1. Two novel variants (c.[253G > A];[938G > A]) in the MTO1 gene were identified in a child with cardiomyopathy with early-onset brain disease. PMID: 29440775
  2. MTO1 deficiency is lethal in some but not all cases, and a genotype-phenotype relation is suggested. Aside from lactic acidosis and cardiomyopathy, developmental delay and other phenotypic features affecting multiple organ systems are often present in these patients, suggesting a broader spectrum than hitherto reported. PMID: 29331171
  3. Defects in the mitochondrial tRNA modification enzymes MTO1 and GTPBP3 promote different metabolic reprogramming through a HIF-PPARgamma-UCP2-AMPK axis. PMID: 29348686
  4. Whole-exome sequencing (WES) indicated a double homozygous mutation in the mitochondrial tRNA translation optimization 1 protein (MTO1) gene (p.R504C and p.V557M) in two of the patients and the homozygous mutation p.R504C in the other. PMID: 27256614
  5. Optic neuropathy, cardiomyopathy, and cognitive disability was found in patients with a homozygous mutation in the nuclear MTO1 and a mitochondrial MT-TF variant. PMID: 26061759
  6. MTO1 mediates tRNA modification and controls mitochondrial translation rate in a highly tissue-specific manner associated with tissue-specific oxidative phosphorylation defects. PMID: 25552653
  7. Results show that post-transcriptional expression of GTPBP3, MTO1 and TRMU genes is down-regulated, leading to mt-tRNA hypomodification and contributing to mitochondrial dysfunction in MELAS cybrids. PMID: 25149473
  8. Nuclear-encoded mitochondrial MTO1 and MRPL41 are regulated in an opposite epigenetic mode based on estrogen receptor status in breast cancer. PMID: 24160266
  9. MTO1 mutations are commonly associated with a presentation of hypertrophic cardiomyopathy, lactic acidosis, and MRC deficiency. PMID: 23929671
  10. MTO1 mutations have roles in hypertrophic cardiomyopathy and lactic acidosis PMID: 22608499
  11. identification of full-length cDNA and elucidation of genomic organization of the human MTO1 homolog PMID: 12011058
  12. Phenotype of non-syndromic deafness associated with the mitochondrial A1555G mutation is modulated by mitochondrial RNA modifying enzymes MTO1 and GTPBP3. PMID: 15542390
  13. proposed linkage in chromosome 8 and the association with TRMU and MTO1 genes were studied in A1555G deafness PMID: 18391568

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Involvement in disease
Combined oxidative phosphorylation deficiency 10 (COXPD10)
Subcellular Location
Protein Families
MnmG family
Tissue Specificity
Ubiquitously expressed in various tissues, but with a markedly elevated expression in tissues of high metabolic rates including cochlea.
Database Links

HGNC: 19261

OMIM: 614667

KEGG: hsa:25821

UniGene: Hs.347614

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