PDHX (NM_003477) Human Untagged Clone

CAT#: SC321608

PDHX (untagged)-Human pyruvate dehydrogenase complex, component X (PDHX), nuclear gene encoding mitochondrial protein, transcript variant 1

Specifications

Product Data

• Type: Human Untagged Clone
• Tag: Tag Free
• Symbol: PDHX
• Synonyms: DLDBP; E3BP; OPDX; PDHXD; PDX1; proX
• Vector: pCMV6-AC
• E. coli Selection: Ampicillin (100 ug/mL)
• Mammalian Cell Selection: Neomycin
• Sequence Data:
  Fully Sequenced ORF

  >OriGene sequence for NM_003477.1 GGGGGGGCAGCCAGTGAGAAGGCCGTCAAGATGGCGGCCTCCTGGAGGCTGGGCTGTGAT
  CCGCGGCTGCTGCGTTATCTTGTGGGCTTCCCTGGCCGCCGAAGCGTAGGGCTGGTGAAG
  GGGGCTCTTGGGTGGTCTGTAAGCCGCGGAGCTAATTGGAGATGGTTTCACAGCACGCAG
  TGGCTTCGGGGTGATCCCATTAAGATACTAATGCCATCACTGTCTCCTACAATGGAAGAA
  GGAAACATTGTGAAATGGCTGAAAAAGGAAGGTGAAGCGGTGAGTGCTGGAGATGCATTA
  TGTGAAATTGAGACTGACAAAGCTGTGGTTACCTTAGATGCAAGTGATGATGGAATCTTG
  GCCAAAATCGTGGTTGAAGAAGGAAGTAAAAATATACGGCTAGGTTCACTAATTGGTTTG
  ATAGTAGAAGAAGGAGAAGATTGGAAACATGTTGAAATTCCCAAAGACGTAGGTCCTCCA
  CCACCAGTTTCAAAACCTTCAGAGCCTCGCCCCTCACCAGAACCACAGATTTCCATCCCT
  GTCAAGAAGGAACACATACCCGGGACACTACGGTTCCGTTTAAGTCCAGCTGCCCGCAAT
  ATTCTGGAAAAACACTCACTGGATGCTAGCCAGGGCACAGCCACTGGCCCTCGGGGGATA
  TTCACTAAAGAGGATGCTCTCAAACTTGTCCAGTTGAAACAAACGGGCAAGATTACCGAG
  TCCAGACCAACTCCAGCCCCCACAGCCACTCCCACAGCACCTTCGCCCCTACAGGCCACA
  GCTGGACCATCTTATCCCCGGCCTGTGATCCCACCAGTATCAACTCCTGGACAACCCAAT
  GCAGTGGGCACATTCACTGAAATCCCCGCCAGCAATATTCGAAGAGTCATTGCCAAGAGA
  TTAACTGAATCTAAAAGTACTGTACCTCATGCATATGCTACTGCTGACTGTGACCTTGGA
  GCTGTTTTAAAAGTTAGGCAAGATCTGGTCAAAGATGACATTAAAGTATCAGTAAATGAT
  TTTATCATCAAGGCAGCAGCTGTTACCCTTAAACAAATGCCAGATGTTAATGTAAGCTGG
  GATGGAGAGGGCCCAAAGCAACTGCCATTTATTGACATTTCAGTGGCTGTGGCAACAGTT
  AAAGGCTTACTTACTCCAATCATAAAAGATGCTGCTGCTAAAGGTATCCAGGAAATTGCT
  GACTCTGTAAAGGCTCTATCAAAGAAAGCAAGAGATGGAAAATTGTTGCCTGAAGAATAC
  CAAGGAGGATCTTTTAGTATTTCCAACTTGGGGATGTTTGGCATCGACGAATTTACTGCA
  GTGATTAACCCTCCTCAGGCCTGCATTTTGGCGGTTGGGAGGTTCCGACCTGTGCTGAAG
  CTCACTGAGGATGAAGAGGGAAATGCCAAACTGCAGCAGCGCCAGCTCATAACAGTCACA
  ATGTCAAGTGACAGTCGAGTGGTTGATGACGAACTGGCAACCAGGTTTCTTAAAAGTTTT
  AAAGCAAACCTAGAGAATCCTATCCGACTTGCCTAGTCCTCAAAGATAAGAAGTTGGTGT
  TCAGCTTAGTTGATTCAGTAGTTGTTACCAAGAAACATATGTTATAGGAAAACAACTTGG
  TATTTAAGTATGAAGTGGATGAAATGTTTATTTATTTAAGGTGAAAGCATTTGACCCAGG
  GTGTCTTCATCTTCAATTTGGGTTTAATGTTATAGAAATAAATGATGATAAACTCTAACT
  AATAAAGGAAAGAGAATATTTGGTTACTCAGATCCATTTTTAACCTCTGGTGCTGTATAA
  AGGGAATATTAAACTAGATGTAAATCAAAGTATATGTTTGGCTCATTTGAGCATTTTGGA
  ATATTTGAGAATGTATGATACATGTAAAATTAAAAAAACTATTAGAACTGTACCATAATT
  ATGTTGAAGGTAGAAGTGATCTTCAAAGAGATGGCCATTAACTTAGCAGTGGGACCTCAC
  TTTTACAAGCACTGCTCTAGATATACTTGAAGAATTTAATATGTACAGAAGTTTATTCTG
  GATAATAAATAAATAAGGATCACACTGTATTAGGGGTTATGGCAACATTATTGAATTTTT
  TATGTACATAAAGCCATATGTTTAGGGTGGTTTCTATCTGTCTTGTTTTTCACTTATATA
  ACACTGTGAACTTCTAAAGAAAGAGGATAAAAGAAGCATGAATGAAAAGAATGACATTCC
  AAAAAAAAAAAAAAAAAAAAAAAAAAAAAA
  5' Read Nucleotide Sequence

  >OriGene 5' read for NM_003477.1 unedited
  GGCGATTGTATACGACTCCTATAGGGCGGCCGGGCAATTCGGCCATTACGGCCGGGGGGG
  CAGCCAGTGAGAAGGCCGTCAAGATGGCGGCCTCCTGGAGGCTGGGCTGTGATCCGCGGC
  TGCTGCGTTATCTTGTGGGCTTCCCTGGCCGCCGAAGCGTAGGGCTGGTGAAGGGGGCTC
  TTGGGTGGTCTGTAAGCCGCGGAGCTAATTGGAGATGGTTTCACAGCACGCAGTGGCTTC
  GGGGTGATCCCATTAAGATACTAATGCCATCACTGTCTCCTACAATGGAAGAAGGAAACA
  TTGTGAAATGGCTGAAAAAGGAAGGTGAAGCGGTGAGTGCTGGAGATGCATTATGTGAAA
  TTGAGACTGACAAAGCTGTGGTTACCTTAGATGCAAGTGATGATGGAATCTTGGCCAAAA
  TCGTGGTTGAAGAAGGAAGTAAAAATATACGGCTAGGTTCACTAATTGGTTTGATAGTAG
  AAGAAGGAGAAGATTGGAAACATGTTGAAATTCCCAAAGACGTAGGTCCTCCACCACCAG
  TTTCAAAACCTTCAGAGCCTCGCCCCTCACCAGAACCACAGATTTCCATCCCTGTCAAGA
  AGAACACATACCCGGGACACTACGGTTCCGTTTAAGTCCAGCTGCCCGCAATATTCTGGA
  AAAACACTCACTGGATGCTAGCCAGGGCACAGCCACTGGCCCTCGGGGGATATTCACTAA
  AGAGGATGCTCTCAAACTTGTCCAGTTGAAACAAACGGGCAAGATTACCGAGTCAGACAA
  CTCAGCCCCCACAGCCACTCCCACAGCACCTTCGCCCTACAGCCACAGCTGACCATCTTA
  TCCCGCTGTGATCCAACAGTATCACTCCTGAACACCATGCGTGGCACATCACTGAAATCC
  CGCCAGCAATATTCGAAGGTCATTGCCAAGAGAGATTAACCTGGA
• Restriction Sites: Please inquire
• ACCN: NM_003477
• ORF Size: 1506 bp
• OTI Disclaimer: Due to the inherent nature of this plasmid, standard methods to replicate additional amounts of DNA in E. coli are highly likely to result in mutations and/or rearrangements. Therefore, OriGene does not guarantee the capability to replicate this plasmid DNA. Additional amounts of DNA can be purchased from OriGene with batch-specific, full-sequence verification at a reduced cost. Please contact our customer care team at custsupport@origene.com or by calling 301.340.3188 option 3 for pricing and delivery.
  
  The molecular sequence of this clone aligns with the gene accession number as a point of reference only. However, individual transcript sequences of the same gene can differ through naturally occurring variations (e.g. polymorphisms), each with its own valid existence. This clone is substantially in agreement with the reference, but a complete review of all prevailing variants is recommended prior to use. More info
• OTI Annotation: This TrueClone is provided through our Custom Cloning Process that includes sub-cloning into OriGene's pCMV6 vector and full sequencing to provide a non-variant match to the expected reference without frameshifts, and is delivered as lyophilized plasmid DNA.

Reference Data

• RefSeq: NM_003477.1, NP_003468.1
• RefSeq Size: 2365
• RefSeq ORF: 1506
• Locus ID: 8050
• Domains: biotin_lipoyl, 2-oxoacid_dh, e3_binding
• Gene Summary: 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]
  Transcript Variant: This variant (1) encodes the longest isoform (1). Sequence Note: Sequence Note: The RefSeq transcript and protein were derived from transcript and genomic sequence to make the sequence consistent with the reference genome assembly. The genomic coordinates used for the transcript record were based on alignments.