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英(yīng)文名稱(chēng)Anti-ALAD/FITC
中(zhōng)文名稱(chēng):FITC標記的δ氨(ān)基(jī)乙(yǐ)酰(xiān)丙(bǐng)酸脫水酶抗(kàng)體(tǐ)
別 名ALAD; ALADH; ALADR; Aminolevulinate dehydratase; Aminolevulinate, delta, dehydratase; Delta aminolevulinic acid dehydratase; Delta-aminolevulinic acid dehydratase; HEM2_HUMAN; Lv; PBGS; Porphobilinogen synthase.
規(guī)格:100ul
說 明 書100ul
研究領域心血(xiě)管 細(xì)胞生物(wù)
抗(kàng)體(tǐ)來源Rabbit
克(kè)隆類(lèi)型Polyclonal
交叉反應(yīng) Human, Mouse, Rat, Dog, Cow, Horse, Rabbit, Sheep,
產品(pǐn)應(yīng)用IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量36kDa
性(xìng) 狀Lyophilized or Liquid
濃 度1mg/ml
免 疫(yì) 原KLH conjugated synthetic peptide derived from human ALAD
亞 型(xíng)IgG
純化(huà)方法affinity purified by Protein A
儲 存 液(yè)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(jiè)紹(shào)background:
Catalyzes an early step in the biosynthesis of tetrapyrroles. Binds two molecules of 5-aminolevulinate per subunit, each at a distinct site, and catalyzes their condensation to form porphobilinogen. Involvement in disease:Defects in ALAD are the cause of acute hepatic porphyria (AHP). AHP is a form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. AHP is characterized by attacks of gastrointestinal disturbances, abdominal colic, paralysis, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors
Function:
Catalyzes an early step in the biosynthesis of tetrapyrroles. Binds two molecules of 5-aminolevulinate per subunit, each at a distinct site, and catalyzes their condensation to form porphobilinogen.
Subunit:
Homooctamer; active form. Homohexamer; low activity form.
DISEASE:
Defects in ALAD are the cause of acute hepatic porphyria (AHEPP) [MIM:612740]. A form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. AHP is characterized by attacks of gastrointestinal disturbances, abdominal colic, paralysis, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors.
Similarity:
Belongs to the ALADH family.
Database links:
UniProtKB/Swiss-Prot: P13716.1
中(zhōng)文名稱(chēng):FITC標記的δ氨(ān)基(jī)乙(yǐ)酰(xiān)丙(bǐng)酸脫水酶抗(kàng)體(tǐ)
別 名ALAD; ALADH; ALADR; Aminolevulinate dehydratase; Aminolevulinate, delta, dehydratase; Delta aminolevulinic acid dehydratase; Delta-aminolevulinic acid dehydratase; HEM2_HUMAN; Lv; PBGS; Porphobilinogen synthase.
詳細介(jiè)紹(shào)：
規(guī)格:100ul
說 明 書100ul
研究領域心血(xiě)管 細(xì)胞生物(wù)
抗(kàng)體(tǐ)來源Rabbit
克(kè)隆類(lèi)型Polyclonal
交叉反應(yīng) Human, Mouse, Rat, Dog, Cow, Horse, Rabbit, Sheep,
產品(pǐn)應(yīng)用IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量36kDa
性(xìng) 狀Lyophilized or Liquid
濃 度1mg/ml
免 疫(yì) 原KLH conjugated synthetic peptide derived from human ALAD
亞 型(xíng)IgG
純化(huà)方法affinity purified by Protein A
儲 存 液(yè)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.
相關(guān)資(zī)料(liào)：
產(chǎn)品介(jiè)紹(shào)background:
Catalyzes an early step in the biosynthesis of tetrapyrroles. Binds two molecules of 5-aminolevulinate per subunit, each at a distinct site, and catalyzes their condensation to form porphobilinogen. Involvement in disease:Defects in ALAD are the cause of acute hepatic porphyria (AHP). AHP is a form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. AHP is characterized by attacks of gastrointestinal disturbances, abdominal colic, paralysis, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors
Function:
Catalyzes an early step in the biosynthesis of tetrapyrroles. Binds two molecules of 5-aminolevulinate per subunit, each at a distinct site, and catalyzes their condensation to form porphobilinogen.
Subunit:
Homooctamer; active form. Homohexamer; low activity form.
DISEASE:
Defects in ALAD are the cause of acute hepatic porphyria (AHEPP) [MIM:612740]. A form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. AHP is characterized by attacks of gastrointestinal disturbances, abdominal colic, paralysis, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors.
Similarity:
Belongs to the ALADH family.
Database links:
UniProtKB/Swiss-Prot: P13716.1
