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Rabbit Anti-DMGDH/PE Conjugated antibody (bs-14345R-PE)
訂購熱線:400-901-9800
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訂購QQ:  400-901-9800
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-14345R-PE
英文名稱1 Rabbit Anti-DMGDH/PE Conjugated antibody
中文名稱 PE標(biāo)記的二甲基甘氨酸脫氫酶抗體
別    名 Dimethylglycine dehydrogenase; Dimethylglycine dehydrogenase, mitochondrial; Dmgdh; M2GD_HUMAN; ME2GLYDH; mitochondrial.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 細(xì)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, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 91kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DMGDH
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產(chǎn)品介紹 background:
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.

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



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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