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  • 丙酮酸脱氢酶 E1 亚基 β(PDHB)基因 | MCE
    PDH 复合物由三种酶成分的多个拷贝组成:丙酮酸脱氢酶 (E1) 、二氢硫辛酰胺乙酰转移酶 (E2) 和硫辛酰胺脱氢酶 (E3) 。 E1 酶是两个 α 和两个 β 亚基的异四聚体。 该基因编码 E1 β 亚基。 该基因的突变与丙酮酸脱氢酶 E1-β 缺乏症有关。 已发现该基因的可变剪接转录物变体。 [RefSeq 提供,2012 年 3 月]
  • 新发现!PDHB 基因突变致丙酮酸脱氢酶缺乏症的致病机制 . . .
    研究人员针对丙酮酸脱氢酶(PDH)缺乏症,研究 PDHB 基因突变,发现其致病且呈常染色体隐性遗传。
  • PDHB基因的意义和作用 - 八方基因
    本文将详细介绍PDHB基因的功能、位置及其在代谢中的重要作用,帮助读者理解这一基因在人体中的关键角色。 什么是PDHB基因? PDHB基因,全名为pyruvate dehydrogenase E1 subunit beta ,位于人类基因组的3号染色体短臂14 3区(3p14 3)。
  • 丙酮酸脱氢酶的结构、功能与相关疾病 - 知乎
    真核PDH以E2和E3BP构成十二面体核心,E1、E3、PDK和PDP结合在外围。 E3与E3BP结合,其它酶与E2结合。 真核PDH之所以如此复杂,是因为它催化三碳向二碳的转化,不仅控制着糖和脂的分界线,也决定氧化代谢还是无氧代谢。 如果PDH被抑制,酵解产生的丙酮酸就不能通过三羧酸循环彻底氧化,只能进行低效的乳酸发酵。 肿瘤细胞的 Warburg效应 就与此有关。 真核生物对PDH的调控主要包括产物抑制、核苷酸反馈调节和共价调节三种。 产物抑制是指乙酰辅酶A抑制E2,NADH抑制E3。 可被辅酶A和NAD+逆转。 核苷酸反馈调节指E1受GTP抑制,被AMP活化。 共价调节是目前的研究热点。 E1上的特殊丝氨酸被PDK磷酸化时失去活性,被PDP水解后恢复活性。
  • 丙酮酸脱氢酶β亚基(Pdhb)通过调节能量供应和基因表达 . . .
    Given the nucleus-localization of Pdhb, further analysis revealed that Pdhb enhances the acetylation of H3K9 and affecting the expression of genes involved in arachidonic acid metabolism and Ras signaling pathway, such as Rsa-14-44 and Pla2g4a, thereby promoting axon regeneration
  • PDHB antibody (14744-1-AP) | Proteintech | 武汉三鹰生物 . . .
    PDHB (Pyruvate dehydrogenase E1 component subunit beta, mitochondrial) is also named as PHE1B The enzyme, which is found in mitochondria, is one of the component enzymes of the pyruvate dehydrogenase multienzyme complex (PDH)
  • Pyruvate dehydrogenase (lipoamide) beta - Wikipedia
    Pyruvate dehydrogenase deficiency is characterized by the buildup of a chemical called lactic acid in the body and a variety of neurological problems Signs and symptoms of this condition usually first appear shortly after birth, and they can vary widely among affected individuals
  • Preparation of monoclonal antibodies against pyruvate dehydrogenase E1 . . .
    In this study, we generated two monoclonal antibodies against the PDHB protein, designated B15–41 and E20–5, and precisely identified their respective B-cell epitopes for the first time: amino acid 10 GALNHA 15 and 52 DQRVWD 57




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