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Rabbit Anti-MSRB3  antibody (bs-17862R)  
~~~促銷代碼KT202411~~~
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產品編號 bs-17862R
英文名稱 Rabbit Anti-MSRB3  antibody
中文名稱 蛋氨酸亞砜還原酶B3抗體
別    名 Deafness, Autosomal Recessive 74; DFNB74; FLJ36866; Methionine R sulfoxide reductase B mitochondrial; Methionine sulfoxide reductase B3; Methionine-R-sulfoxide reductase B3; MsrB3; Msrb3; MSRB3_HUMAN.  
研究領域 細胞生物  免疫學  信號轉導  新陳代謝  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human,Mouse,Rat,Rabbit,Pig,Sheep,Cow,Chicken,Dog,Horse)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 173kDa
細胞定位 細胞漿 線粒體
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MSRB3: 33-120/192 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 The protein encoded by this gene catalyzes the reduction of methionine sulfoxide to methionine. This enzyme acts as a monomer and requires zinc as a cofactor. Several transcript variants encoding two different isoforms have been found for this gene. One of the isoforms localizes to mitochondria while the other localizes to endoplasmic reticula. [provided by RefSeq, Jul 2010]

Function:
Catalyzes the reduction of free and protein-bound methionine sulfoxide to methionine. Isoform 2 is essential for hearing.

Subcellular Location:
Mitochondrion and Endoplasmic reticulum.

Tissue Specificity:
Widely expressed.

DISEASE:
Deafness, autosomal recessive, 74 (DFNB74) [MIM:613718]: A form of non-syndromic sensorineural deafness characterized by prelingual, bilateral, profound hearing loss. Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information. Note=The disease is caused by mutations affecting the gene represented in this entry. A nonsense mutation affecting exclusively mitochondrial isoform 2 is sufficient to produce hearing loss.

Similarity:
Belongs to the MsrB Met sulfoxide reductase family.

SWISS:
Q8IXL7

Gene ID:
253827

Database links:

Entrez Gene: 253827 Human

Entrez Gene: 320183 Mouse

Entrez Gene: 680036 Rat

Omim: 613719 Human

SwissProt: Q8IXL7 Human

SwissProt: Q8BU85 Mouse

Unigene: 339024 Human



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