人 ALDOB 抗体对
01AB01624
Aldolase, fructose-bisphosphate B (ALDOB) is a cytosolic homotetrameric enzyme predominantly expressed in the liver, kidney, and small intestine, where it is essential for dietary fructose metabolism. It catalyzes the reversible cleavage of fructose-1,6-bisphosphate and fructose-1-phosphate into dihydroxyacetone phosphate and glyceraldehyde or glyceraldehyde-3-phosphate. The catalytic mechanism involves a Schiff base intermediate formed by a conserved lysine residue within its alpha/beta barrel structure. Clinically, pathogenic variants in the ALDOB gene cause hereditary fructose intolerance (HFI), an autosomal recessive metabolic disorder. HFI is characterized by the toxic accumulation of fructose-1-phosphate, leading to severe hypoglycemia, hepatic and renal dysfunction, and systemic symptoms upon the ingestion of fructose, sucrose, or sorbitol.
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技术参数
- 靶点基因名
- ALDOB
- 基因别名
- ALDB
- 蛋白别名
- aldolase, fructose-bisphosphate B; ALDOB
- UniProt
- P05062
- 物种
- Human
产品详情
- 靶点名称
- ALDOB
- 蛋白名称
- aldolase, fructose-bisphosphate B
- 蛋白别名
- Fructose-bisphosphate aldolase B; Liver-type aldolase
- 靶点基因名
- ALDOB
- 基因别名
- ALDB
- UniProt
- P05062
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Aldolase, fructose-bisphosphate B (ALDOB) is a cytosolic homotetrameric enzyme predominantly expressed in the liver, kidney, and small intestine, where it is essential for dietary fructose metabolism. It catalyzes the reversible cleavage of fructose-1,6-bisphosphate and fructose-1-phosphate into dihydroxyacetone phosphate and glyceraldehyde or glyceraldehyde-3-phosphate. The catalytic mechanism involves a Schiff base intermediate formed by a conserved lysine residue within its alpha/beta barrel structure. Clinically, pathogenic variants in the ALDOB gene cause hereditary fructose intolerance (HFI), an autosomal recessive metabolic disorder. HFI is characterized by the toxic accumulation of fructose-1-phosphate, leading to severe hypoglycemia, hepatic and renal dysfunction, and systemic symptoms upon the ingestion of fructose, sucrose, or sorbitol.