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Product Name Anti-TRIM37/PE-Cy3 Chinese Name PE-Cy3标记的TRIM37蛋白抗体 Alias E3 ubiquitin protein ligase TRIM37; E3 ubiquitin-protein ligase TRIM37; KIAA0898; MUL; MUL protein; Mulibrey nanism gene; Mulibrey nanism protein; POB 1; POB1; RING B box coiled coil protein; TEF 3; TEF3; TRI37_HUMAN; TRIM 37; Trim37; Tripartite motif containing 37; Tripartite motif containing 37 protein; Tripartite motif containing protein 37; Tripartite motif-containing protein 37. Research Area Cell biology transcriptional regulatory factor Epigenetics Ubiquitin Immunogen Species Rabbit Clonality Polyclonal React Species (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, ) 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 108kDa Form Lyophilized or Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human TRIM37 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:
TRIM37 is a protein that localizes to peroxisomes and contains a tripartite motif (TRIM) and a tumor necrosis factor-receptor associated factor (TRAF) domain. The protein and gene forms of TRIM37 are highly conserved between human and mouse. TRIM37 is expressed at a low level in the liver, ovary, heart, lung, skeletal muscle, and kidney, while it is highly expressed in the testis and brain, where it may act as an E3 ubiquitin ligase. Mutations in the TRIM37 gene result in Mulibrey nanism, an autosomal recessive prenatal-onset growth disorder that causes characteristic dysmorphic craniofacial features, heart disease, cardiopathy, failure of sexual maturation, and hepatomegaly.
Function:
E3 ubiquitin-protein ligase.
Subcellular Location:
Cytoplasm > perinuclear region. Peroxisome. Found in vesicles of the peroxisome. Aggregates as aggresomes, a perinuclear region where certain misfolded or aggregated proteins are sequestered for proteasomal degradation.
Tissue Specificity:
Ubiquitous.
Post-translational modifications:
Auto-ubiquitinated.
DISEASE:
Defects in TRIM37 are the cause of mulibrey nanism (MUL) [MIM:253250]; also known as muscle-liver-brain-eye nanism. MUL is an autosomal recessive disorder that involves several tissues of mesodermal origin, implying a defect in a highly pleiotropic gene. Characteristic features include severe growth failure of prenatal onset and constrictive pericardium with consequent hepatomegaly. In addition, muscle hypotonia, J-shaped sella turcica, yellowish dots in the ocular fundi, typical dysmorphic features and hypoplasia of various endocrine glands causing hormonal deficiency are common.
Similarity:
Belongs to the TRIM/RBCC family.
Contains 1 B box-type zinc finger.
Contains 1 MATH domain.
Contains 1 RING-type zinc finger.
Database links:Entrez Gene: 4591 Human
Omim: 605073 Human
SwissProt: O94972 Human
Unigene: 579079 Human
Unigene: 605697 Human
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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