• 商品货号：F111805

• accession：NM_003477
• 基因别名：PDHX
• 基因描述：Homo sapiens pyruvate dehydrogenase complex, component X (PDHX), transcript variant 1, mRNA.
• 载体： 现货载体
• CDS区长度：1506
• 翻译后氨基酸长度：501
• TranscriptVariant：This variant (1) encodes the longest isoform (1).
• 基因简介：The pyruvate dehydrogenase (PDH) complex is located in the mitochondrial matrix and catalyzes the conversion of pyruvate to acetyl coenzyme A. The PDH complex thereby links glycolysis to Krebs cycle. The PDH complex contains three catalytic subunits, E1, E2, and E3, two regulatory subunits, E1 kinase and E1 phosphatase, and a non-catalytic subunit, E3 binding protein (E3BP). This gene encodes the E3 binding protein subunit; also known as component X of the pyruvate dehydrogenase complex. This protein tethers E3 dimers to the E2 core of the PDH complex. Defects in this gene are a cause of pyruvate dehydrogenase deficiency which results in neurological dysfunction and lactic acidosis in infancy and early childhood. This protein is also a minor antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC eventually leads to cirrhosis and liver failure. Alternative splicing results in multiple transcript variants encoding distinct isoforms.[provided by RefSeq, Oct 2009]
• 载体信息：自有图片地址
• 规格：10ul 质粒

CDS区参考序列： 点击查看序列

ATGGCGGCCTCCTGGAGGCTGGGCTGTGATCCGCGGCTGCTGCGTTATCTTGTGGGCTTCCCCGGCCGCCGAAGCGTAGGGCTGGTGAAGGGGGCTCTTGGGTGGTCTGTAAGCCGCGGAGCTAATTGGAGATGGTTTCACAGCACGCAGTGGCTTCGGGGTGATCCCATTAAGATACTAATGCCATCACTGTCTCCTACAATGGAAGAAGGAAACATTGTGAAATGGCTGAAAAAGGAAGGTGAAGCGGTGAGTGCTGGAGATGCATTATGTGAAATTGAGACTGACAAAGCTGTGGTTACCTTAGATGCAAGTGATGATGGAATCTTGGCCAAAATCGTGGTTGAAGAAGGAAGTAAAAATATACGGCTAGGTTCACTAATTGGTTTGATAGTAGAAGAAGGAGAAGATTGGAAACATGTTGAAATTCCCAAAGACGTAGGTCCTCCACCACCAGTTTCAAAACCTTCAGAGCCTCGCCCCTCACCAGAACCACAGATTTCCATCCCTGTCAAGAAGGAACACATACCCGGGACACTACGGTTCCGTTTAAGTCCAGCTGCCCGCAATATTCTGGAAAAACACTCACTGGATGCTAGCCAGGGCACAGCCACTGGCCCTCGGGGGATATTCACTAAAGAGGATGCTCTCAAACTTGTCCAGTTGAAACAAACGGGCAAGATTACCGAGTCCAGACCAACTCCAGCCCCCACAGCCACTCCCACAGCACCTTCGCCCCTACAGGCCACAGCTGGACCATCTTATCCCCGGCCTGTGATCCCACCAGTATCAACTCCTGGACAACCCAATGCAGTGGGCACATTCACTGAAATCCCCGCCAGCAATATTCGAAGAGTTATTGCCAAGAGATTAACTGAATCTAAAAGTACTGTACCTCATGCATATGCTACTGCTGACTGTGACCTTGGAGCTGTTTTAAAAGTTAGGCAAGATCTGGTCAAAGATGACATTAAAGTATCAGTAAATGATTTTATCATCAAGGCAGCAGCTGTTACCCTTAAACAAATGCCAGATGTTAATGTAAGCTGGGATGGAGAGGGCCCAAAGCAACTGCCATTTATTGACATTTCAGTGGCTGTGGCAACAGATAAAGGCTTACTTACTCCAATCATAAAAGATGCTGCTGCTAAAGGTATCCAGGAAATTGCTGACTCTGTAAAGGCTCTATCAAAGAAAGCAAGAGATGGAAAATTGTTGCCTGAAGAATACCAAGGAGGATCTTTTAGTATTTCCAACTTGGGGATGTTTGGCATCGACGAATTTACTGCAGTGATTAACCCTCCTCAGGCCTGCATTTTGGCGGTTGGGAGGTTCCGACCTGTGCTGAAGCTCACTGAGGATGAAGAGGGAAATGCCAAACTGCAGCAGCGCCAGCTCATAACAGTCACAATGTCAAGTGACAGTCGAGTGGTTGATGACGAACTGGCAACCAGGTTTCTTAAAAGTTTTAAAGCAAACCTAGAGAATCCTATCCGACTTGCCTAG

翻译后氨基酸参数序列： 点击查看序列

ATGGCGGCCTCCTGGAGGCTGGGCTGTGATCCGCGGCTGCTGCGTTATCTTGTGGGCTTCCCCGGCCGCCGAAGCGTAGGGCTGGTGAAGGGGGCTCTTGGGTGGTCTGTAAGCCGCGGAGCTAATTGGAGATGGTTTCACAGCACGCAGTGGCTTCGGGGTGATCCCATTAAGATACTAATGCCATCACTGTCTCCTACAATGGAAGAAGGAAACATTGTGAAATGGCTGAAAAAGGAAGGTGAAGCGGTGAGTGCTGGAGATGCATTATGTGAAATTGAGACTGACAAAGCTGTGGTTACCTTAGATGCAAGTGATGATGGAATCTTGGCCAAAATCGTGGTTGAAGAAGGAAGTAAAAATATACGGCTAGGTTCACTAATTGGTTTGATAGTAGAAGAAGGAGAAGATTGGAAACATGTTGAAATTCCCAAAGACGTAGGTCCTCCACCACCAGTTTCAAAACCTTCAGAGCCTCGCCCCTCACCAGAACCACAGATTTCCATCCCTGTCAAGAAGGAACACATACCCGGGACACTACGGTTCCGTTTAAGTCCAGCTGCCCGCAATATTCTGGAAAAACACTCACTGGATGCTAGCCAGGGCACAGCCACTGGCCCTCGGGGGATATTCACTAAAGAGGATGCTCTCAAACTTGTCCAGTTGAAACAAACGGGCAAGATTACCGAGTCCAGACCAACTCCAGCCCCCACAGCCACTCCCACAGCACCTTCGCCCCTACAGGCCACAGCTGGACCATCTTATCCCCGGCCTGTGATCCCACCAGTATCAACTCCTGGACAACCCAATGCAGTGGGCACATTCACTGAAATCCCCGCCAGCAATATTCGAAGAGTTATTGCCAAGAGATTAACTGAATCTAAAAGTACTGTACCTCATGCATATGCTACTGCTGACTGTGACCTTGGAGCTGTTTTAAAAGTTAGGCAAGATCTGGTCAAAGATGACATTAAAGTATCAGTAAATGATTTTATCATCAAGGCAGCAGCTGTTACCCTTAAACAAATGCCAGATGTTAATGTAAGCTGGGATGGAGAGGGCCCAAAGCAACTGCCATTTATTGACATTTCAGTGGCTGTGGCAACAGATAAAGGCTTACTTACTCCAATCATAAAAGATGCTGCTGCTAAAGGTATCCAGGAAATTGCTGACTCTGTAAAGGCTCTATCAAAGAAAGCAAGAGATGGAAAATTGTTGCCTGAAGAATACCAAGGAGGATCTTTTAGTATTTCCAACTTGGGGATGTTTGGCATCGACGAATTTACTGCAGTGATTAACCCTCCTCAGGCCTGCATTTTGGCGGTTGGGAGGTTCCGACCTGTGCTGAAGCTCACTGAGGATGAAGAGGGAAATGCCAAACTGCAGCAGCGCCAGCTCATAACAGTCACAATGTCAAGTGACAGTCGAGTGGTTGATGACGAACTGGCAACCAGGTTTCTTAAAAGTTTTAAAGCAAACCTAGAGAATCCTATCCGACTTGCCTAG