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英文名稱Anti-AASS/FITC
中文名稱:FITC標記的賴氨酸酮戊二酸還原酶抗體
別 名Alpha aminoadipic semialdehyde synthase mitochondrial; LKR/SDH; LKRSDH; LORSDH; Lysine ketoglutarate reductase; Saccharopine dehydrogenase; AASS_HUMAN.
規格:100ul
說 明 書100ul
研究領域腫瘤 細胞生物 信號轉導
抗體來源Rabbit
克隆類型Polyclonal
交叉反應 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
產品應用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99kDa
性 狀Lyophilized or Liquid
濃 度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human LKRSDH (878-926aa)
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.
產品介紹background:
Alpha-aminoadipic semialdehyde synthase (AASS), also designated lysine ketoglutarate reductase (LKR) or saccharopine dehydrogenase (SDH), is a 926 amino acid protein that exists as a homodimer in the mitochondria. AASS acts as a bifunctional enzyme containing the lysine alpha-ketoglutarate reductase (LKR) and saccharopine dehydrogenase activities that catalyzes the first two steps in lysine degradation. It is widely expressed with highest expression in liver and transcription of the AASS gene is induced upon starvation. Mutations in the gene encoding AASS result in various forms familial hyperlysinemias (FH), autosomal recessive disorders characterized by hyperlysinemia, lysinuria, and variable saccharopinuria. However, no adverse mental or physical effects have been found in patients with hyperlysinemia
Function:
Bifunctional enzyme that catalyzes the first two steps in lysine degradation. The N-terminal and the C-terminal contain lysine-ketoglutarate reductase and saccharopine dehydrogenase activity, respectively.
Subunit:
Homodimer
Subcellular Location:
Mitochondrial
Tissue Specificity:
Expressed in all 16 tissues examined with highest expression in the liver.
DISEASE:
Defects in AASS are the cause of hyperlysinemia (HYPLYS) [MIM:238700]. Hyperlysinemia is an autosomal recessive condition characterized by hyperlysinemia lysinuria and variable saccharopinuria.
中文名稱:FITC標記的賴氨酸酮戊二酸還原酶抗體
別 名Alpha aminoadipic semialdehyde synthase mitochondrial; LKR/SDH; LKRSDH; LORSDH; Lysine ketoglutarate reductase; Saccharopine dehydrogenase; AASS_HUMAN.
詳細介紹：
規格:100ul
說 明 書100ul
研究領域腫瘤 細胞生物 信號轉導
抗體來源Rabbit
克隆類型Polyclonal
交叉反應 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
產品應用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99kDa
性 狀Lyophilized or Liquid
濃 度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human LKRSDH (878-926aa)
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.
相關資料：
產品介紹background:
Alpha-aminoadipic semialdehyde synthase (AASS), also designated lysine ketoglutarate reductase (LKR) or saccharopine dehydrogenase (SDH), is a 926 amino acid protein that exists as a homodimer in the mitochondria. AASS acts as a bifunctional enzyme containing the lysine alpha-ketoglutarate reductase (LKR) and saccharopine dehydrogenase activities that catalyzes the first two steps in lysine degradation. It is widely expressed with highest expression in liver and transcription of the AASS gene is induced upon starvation. Mutations in the gene encoding AASS result in various forms familial hyperlysinemias (FH), autosomal recessive disorders characterized by hyperlysinemia, lysinuria, and variable saccharopinuria. However, no adverse mental or physical effects have been found in patients with hyperlysinemia
Function:
Bifunctional enzyme that catalyzes the first two steps in lysine degradation. The N-terminal and the C-terminal contain lysine-ketoglutarate reductase and saccharopine dehydrogenase activity, respectively.
Subunit:
Homodimer
Subcellular Location:
Mitochondrial
Tissue Specificity:
Expressed in all 16 tissues examined with highest expression in the liver.
DISEASE:
Defects in AASS are the cause of hyperlysinemia (HYPLYS) [MIM:238700]. Hyperlysinemia is an autosomal recessive condition characterized by hyperlysinemia lysinuria and variable saccharopinuria.
