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Rabbit Anti-DMGDH  antibody (bs-14345R)  
~~~促銷代碼KT202411~~~
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產(chǎn)品編號 bs-14345R
英文名稱 Rabbit Anti-DMGDH  antibody
中文名稱 二甲基甘氨酸脫氫酶抗體
別    名 Dimethylglycine dehydrogenase; Dimethylglycine dehydrogenase, mitochondrial; Dmgdh; M2GD_HUMAN; ME2GLYDH; mitochondrial.  
研究領(lǐng)域 細胞生物  神經(jīng)生物學(xué)  信號轉(zhuǎn)導(dǎo)  新陳代謝  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human,Mouse,Rat,Rabbit,Pig,Sheep,Cow,Dog,Horse)
產(chǎn)品應(yīng)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 91kDa
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 DMGDH: 101-200/866 
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(chǎn)品介紹 This gene encodes an enzyme involved in the catabolism of choline, catalyzing the oxidative demethylation of dimethylglycine to form sarcosine. The enzyme is found as a monomer in the mitochondrial matrix, and uses flavin adenine dinucleotide and folate as cofactors. Mutation in this gene causes dimethylglycine dehydrogenase deficiency, characterized by a fishlike body odor, chronic muscle fatigue, and elevated levels of the muscle form of creatine kinase in serum. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2013]

Subcellular Location:
Mitochondrion.

DISEASE:
Defects in DMGDH are the cause of DMGDH deficiency (DMGDHD) [MIM:605850]. DMGDHD is a disorder characterized by fish odor, muscle fatigue with increased serum creatine kinase. Biochemically it is characterized by an increase of N,N-dimethylglycine (DMG) in serum and urine.

Similarity:
Belongs to the GcvT family.

SWISS:
Q9UI17

Gene ID:
29958

Database links:

Entrez Gene: 29958 Human

Entrez Gene: 504453 Cow

Entrez Gene: 488935 Dog

Entrez Gene: 100724353 Guinea pig

Entrez Gene: 100073267 Horse

Entrez Gene: 74129 Mouse

Entrez Gene: 100525563 Pig

Entrez Gene: 100340207 Rabbit

Entrez Gene: 245961 Rat

Omim: 605849 Human

SwissProt: Q9UI17 Human

SwissProt: Q9DBT9 Mouse

SwissProt: Q63342 Rat

Unigene: 655653 Human

Unigene: 21789 Mouse

Unigene: 3646 Rat



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