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Product Name Anti-TMEM70/RBITC Chinese Name 罗丹明(RBITC)标记的Transmembrane protein70抗体 Alias FLJ20533; MC5DN2; mitochondrial; OTTHUMP00000226855; OTTHUMP00000226856; TMEM70; TMM70_HUMAN; Transmembrane protein 70; Transmembrane protein 70 mitochondrial; Transmembrane protein 70, mitochondrial. Research Area Tumour Cell biology Signal transduction Transmembrane protein Immunogen Species Rabbit Clonality Polyclonal React Species (predicted: Human, ) Applications ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Molecular weight 21kDa Form Lyophilized or Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human TMEM70 Lsotype IgG Purification affinity purified by Protein A Storage Buffer 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. Product Detail background:
This gene likely encodes a mitochondrial membrane protein. The encoded protein may play a role in biogenesis of mitochondrial ATP synthase. Mutations in this gene have been associated with neonatal mitochondrial encephalocardiomyopathy due to ATP synthase deficiency. Alternatively spliced transcript variants have been described. [provided by RefSeq, Feb 2010]
Function:
Involved in biogenesis of mitochondrial ATP synthase.
Subcellular Location:
Mitochondrion inner membrane.
DISEASE:
Defects in TMEM70 are a cause of mitochondrial encephalocardiomyopathy neonatal due to ATP synthase deficiency (MT-ATPSD) [MIM:604273]; also known as ATPase deficiency. A mitochondrial disorder with heterogeneous clinical manifestations including dysmorphic features, psychomotor retardation, hypotonia, growth retardation, cardiomyopathy, enlarged liver, hypoplastic kidneys and elevated lactate levels in urine, plasma and cerebrospinal fluid.
Similarity:
Belongs to the TMEM70 family.
Database links:Entrez Gene: 54968 Human
Entrez Gene: 70397 Mouse
Omim: 612418 Human
SwissProt: Q9BUB7 Human
SwissProt: Q921N7 Mouse
Unigene: 106650 Human
Unigene: 194225 Mouse
Unigene: 22296 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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