推荐产品
生物源
rabbit
品質等級
共軛
unconjugated
抗體表格
affinity isolated antibody
抗體產品種類
primary antibodies
無性繁殖
polyclonal
產品線
Prestige Antibodies® Powered by Atlas Antibodies
形狀
buffered aqueous glycerol solution
物種活性
human
技術
immunoblotting: 0.04-0.4 μg/mL
immunohistochemistry: 1:20-1:50
免疫原序列
RVIGNMALNEHLHSSIVRSGWVSIMAEAMKSPHIMESSHAARILANLDRETVQEKYQDGVYVLHPQYRTSQPIKA
UniProt登錄號
運輸包裝
wet ice
儲存溫度
−20°C
目標翻譯後修改
unmodified
基因資訊
human ... SERAC1(84947)
一般說明
The gene SERAC1 (serine active site containing 1) is mapped to human chromosome 6q25.3. The protein has a serine-lipase domain and is present at the contact areas between mitochondria and endoplasmic reticulum.
免疫原
Protein SERAC1 recombinant protein epitope signature tag (PrEST)
應用
All Prestige Antibodies®Powered by Atlas Antibodies is developed and validated by the Human Protein Atlas (HPA) project . Each antibody is tested by immunohistochemistry against hundreds of normal and disease tissues. These images can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
Anti-SERAC1 antibody produced in rabbit has been used for western blotting and immunofluorescence.
Anti-SERAC1 antibody produced in rabbit has been used for western blotting and immunofluorescence.
生化／生理作用
SERAC1 (serine active site containing 1) might be involved in phosphatidylglycerol remodeling pathways. It is required for proper mitochondrial functioning and cholesterol transport. Mutations in the gene are associated with infantile mitochondrial hepatopathy, a characteristic of MEGDEL syndrome (3-methylglutaconic aciduria type IV with sensorineural deafness, encephalopathy and Leigh-like syndrome).
特點和優勢
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
Every Prestige Antibody is tested in the following ways:
- IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
- Protein array of 364 human recombinant protein fragments.
聯結
Corresponding Antigen APREST77427
外觀
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
法律資訊
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
免責聲明
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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儲存類別代碼
10 - Combustible liquids
水污染物質分類（WGK）
WGK 1
閃點（°F）
Not applicable
閃點（°C）
Not applicable
Vineta Fellman et al.
Biochimica et biophysica acta. Molecular basis of disease, 1868(1), 166298-166298 (2021-11-10)
In the diagnostic work-up of a newborn infant with a metabolic crisis, lethal multiorgan failure on day six of life, and increased excretion of 3-methylglutaconic acid, we found using whole genome sequencing a homozygous SERAC1 mutation indicating MEGDHEL syndrome (3-methylglutaconic
Mutations in the phospholipid remodeling gene SERAC1 impair mitochondrial function and intracellular cholesterol trafficking and cause dystonia and deafness.
Wortmann SB, et. al.
Nature Genetics, 44, 797-802 (2012)
Nuclear factors: roles related to mitochondrial deafness.
Luo LF
Gene, 520, 79-89 (2013)
Saskia B Wortmann et al.
Nature genetics, 44(7), 797-802 (2012-06-12)
Using exome sequencing, we identify SERAC1 mutations as the cause of MEGDEL syndrome, a recessive disorder of dystonia and deafness with Leigh-like syndrome, impaired oxidative phosphorylation and 3-methylglutaconic aciduria. We localized SERAC1 at the interface between the mitochondria and the
Infantile mitochondrial hepatopathy is a cardinal feature of MEGDEL syndrome (3-methylglutaconic aciduria type IV with sensorineural deafness, encephalopathy and Leigh-like syndrome) caused by novel mutations in SERAC1.
Sarig O
American Journal of Medical Genetics. Part A, 161A, 2204-2215 (2013)
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