產(chǎn)品中(zhōng)心
標(biāo)記(jì)一抗
英文(wén)名稱Anti-ACADL/FITC
中文(wén)名(míng)稱(chēng):FITC標記(jì)的酰基輔酶A脫(tuō)氫(qīng)酶(méi)長鏈抗體(tǐ)
別(bié) 名mitochondrial; ACAD4; ACADL; ACADL_HUMAN; Acyl Coenzyme A dehydrogenase long chain; FLJ94052; LCAD; Long chain acyl CoA dehydrogenase; Long-chain specific acyl-CoA dehydrogenase.
規格:100ul
說 明(míng) 書100ul
研(yán)究領域腫(zhǒng)瘤(liú) 細胞生物 免(miǎn)疫(yì)學 轉錄調(tiáo)節因(yīn)子(zǐ) 綫粒(lì)體(tǐ)
抗(kàng)體來源(yuán)Rabbit
克(kè)隆類型Polyclonal
交叉(chā)反應 Human, Mouse, Rat,
產品(pǐn)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44kDa
性(xìng) 狀Lyophilized or Liquid
濃 度1mg/ml
免(miǎn) 疫 原(yuán)KLH conjugated synthetic peptide derived from human ACADL
亞 型IgG
純(chún)化(huà)方(fāng)法affinity purified by Protein A
儲(chǔ) 存(cún) 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保(bǎo)存(cún)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.
產品介(jiè)紹background:
The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. This protein is one of the four enzymes that catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia. [provided by RefSeq]
Subunit:
Homotetramer.
Subcellular Location:
Mitochondrion matrix.
DISEASE:
Defects in ACADL are a cause of acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD) [MIM:201475]. An inborn error of mitochondrial fatty acid beta-oxidation which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form characterized by early onset, high mortality and high incidence of cardiomyopathy; a milder childhood form with later onset, characterized by hypoketotic hypoglycemia, low mortality and rare cardiomyopathy; an adult form, with isolated skeletal muscle involvement, rhabdomyolysis and myoglobinuria, usually triggered by exercise or fasting.
中文(wén)名(míng)稱(chēng):FITC標記(jì)的酰基輔酶A脫(tuō)氫(qīng)酶(méi)長鏈抗體(tǐ)
別(bié) 名mitochondrial; ACAD4; ACADL; ACADL_HUMAN; Acyl Coenzyme A dehydrogenase long chain; FLJ94052; LCAD; Long chain acyl CoA dehydrogenase; Long-chain specific acyl-CoA dehydrogenase.
詳細介(jiè)紹(shào)：
規格:100ul
說 明(míng) 書100ul
研(yán)究領域腫(zhǒng)瘤(liú) 細胞生物 免(miǎn)疫(yì)學 轉錄調(tiáo)節因(yīn)子(zǐ) 綫粒(lì)體(tǐ)
抗(kàng)體來源(yuán)Rabbit
克(kè)隆類型Polyclonal
交叉(chā)反應 Human, Mouse, Rat,
產品(pǐn)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44kDa
性(xìng) 狀Lyophilized or Liquid
濃 度1mg/ml
免(miǎn) 疫 原(yuán)KLH conjugated synthetic peptide derived from human ACADL
亞 型IgG
純(chún)化(huà)方(fāng)法affinity purified by Protein A
儲(chǔ) 存(cún) 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保(bǎo)存(cún)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.
相關(guān)資料：
產品介(jiè)紹background:
The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. This protein is one of the four enzymes that catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia. [provided by RefSeq]
Subunit:
Homotetramer.
Subcellular Location:
Mitochondrion matrix.
DISEASE:
Defects in ACADL are a cause of acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD) [MIM:201475]. An inborn error of mitochondrial fatty acid beta-oxidation which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form characterized by early onset, high mortality and high incidence of cardiomyopathy; a milder childhood form with later onset, characterized by hypoketotic hypoglycemia, low mortality and rare cardiomyopathy; an adult form, with isolated skeletal muscle involvement, rhabdomyolysis and myoglobinuria, usually triggered by exercise or fasting.
- 上一條：FITC標記的肌萎(wěi)縮(suō)側索硬化蛋白(bái)2抗體
- 下一(yī)條(tiáo)：FITC標(biāo)記(jì)的脂肪(fáng)醛脫(tuō)氫酶(méi)抗體
