MTO1 Antibody

Code CSB-PA846064ESR2HU
Size US$166
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  • Western blot
    All lanes: MTO1 antibody at 2.25µg/ml
    Lane 1: Rat heart tissue
    Lane 2: Jurkat whole cell lysate
    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 brain tissue using CSB-PA846064ESR2HU at dilution of 1:100

  • Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA846064ESR2HU at dilution of 1:100

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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) MTO1 Polyclonal antibody
Uniprot No.
Target Names
Alternative Names
MTO1 antibody; CGI-02 antibody; Protein MTO1 homolog antibody; mitochondrial antibody
Raised in
Species Reactivity
Human, Rat
Recombinant Human Protein MTO1 homolog, mitochondrial protein (420-680AA)
Immunogen Species
Homo sapiens (Human)
Purification Method
Antigen Affinity Purified
It differs from different batches. Please contact us to confirm it.
PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Tested Applications
Recommended Dilution
Application Recommended Dilution
WB 1:1000-1:5000
IHC 1:20-1:200
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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