Details for: PDHA1

Gene ID: 5160

Symbol: PDHA1

Ensembl ID: ENSG00000131828

Description: pyruvate dehydrogenase E1 subunit alpha 1

Associated with

Cells (max top 100)

(Cell Significance Index and respective Thresholds are uniquely calculated using our advanced thresholding algorithms to reveal cell-specific gene markers)

  • Cell Name: polychromatophilic erythroblast (CL0000550)
    Fold Change: 176.0260
    Cell Significance Index: -27.3800
  • Cell Name: hematopoietic oligopotent progenitor cell (CL0002032)
    Fold Change: 116.1867
    Cell Significance Index: -29.4700
  • Cell Name: embryonic stem cell (CL0002322)
    Fold Change: 97.0512
    Cell Significance Index: -39.9800
  • Cell Name: smooth muscle fiber of ileum (CL1000278)
    Fold Change: 86.7995
    Cell Significance Index: -40.9800
  • Cell Name: mucosal type mast cell (CL0000485)
    Fold Change: 86.0170
    Cell Significance Index: -34.9500
  • Cell Name: peripheral blood mononuclear cell (CL2000001)
    Fold Change: 77.4614
    Cell Significance Index: -39.8500
  • Cell Name: ileal goblet cell (CL1000326)
    Fold Change: 61.2051
    Cell Significance Index: -41.0700
  • Cell Name: ciliated cell of the bronchus (CL0002332)
    Fold Change: 36.9209
    Cell Significance Index: -35.2500
  • Cell Name: orthochromatic erythroblast (CL0000552)
    Fold Change: 30.0822
    Cell Significance Index: -37.0900
  • Cell Name: CD8-alpha-beta-positive, alpha-beta intraepithelial T cell (CL0000796)
    Fold Change: 12.1264
    Cell Significance Index: -32.4900
  • Cell Name: stromal cell of bone marrow (CL0010001)
    Fold Change: 9.9644
    Cell Significance Index: -39.3200
  • Cell Name: CD8-positive, alpha-beta regulatory T cell (CL0000795)
    Fold Change: 8.9632
    Cell Significance Index: -27.5300
  • Cell Name: gut absorptive cell (CL0000677)
    Fold Change: 2.9035
    Cell Significance Index: 174.3100
  • Cell Name: enterocyte of epithelium of large intestine (CL0002071)
    Fold Change: 1.6750
    Cell Significance Index: 75.9200
  • Cell Name: epithelial cell of small intestine (CL0002254)
    Fold Change: 1.6119
    Cell Significance Index: 262.1600
  • Cell Name: acinar cell of salivary gland (CL0002623)
    Fold Change: 1.5663
    Cell Significance Index: 73.0300
  • Cell Name: basal cell of prostate epithelium (CL0002341)
    Fold Change: 1.5067
    Cell Significance Index: 41.0100
  • Cell Name: conjunctival epithelial cell (CL1000432)
    Fold Change: 1.3889
    Cell Significance Index: 18.9500
  • Cell Name: eye photoreceptor cell (CL0000287)
    Fold Change: 1.1181
    Cell Significance Index: 70.4700
  • Cell Name: intermediate cell of urothelium (CL4030055)
    Fold Change: 0.9124
    Cell Significance Index: 164.4900
  • Cell Name: bladder urothelial cell (CL1001428)
    Fold Change: 0.9098
    Cell Significance Index: 47.2600
  • Cell Name: sebum secreting cell (CL0000317)
    Fold Change: 0.9046
    Cell Significance Index: 63.9800
  • Cell Name: basal cell of urothelium (CL1000486)
    Fold Change: 0.8918
    Cell Significance Index: 109.6600
  • Cell Name: neutrophil progenitor cell (CL0000834)
    Fold Change: 0.8647
    Cell Significance Index: 23.1300
  • Cell Name: L2/3-6 intratelencephalic projecting glutamatergic neuron (CL4023040)
    Fold Change: 0.7367
    Cell Significance Index: 147.7800
  • Cell Name: glycinergic neuron (CL1001509)
    Fold Change: 0.7205
    Cell Significance Index: 37.8300
  • Cell Name: mesonephric nephron tubule epithelial cell (CL1000022)
    Fold Change: 0.6760
    Cell Significance Index: 23.4900
  • Cell Name: luminal adaptive secretory precursor cell of mammary gland (CL4033057)
    Fold Change: 0.6353
    Cell Significance Index: 29.8600
  • Cell Name: stromal cell of ovary (CL0002132)
    Fold Change: 0.6308
    Cell Significance Index: 86.6200
  • Cell Name: skeletal muscle myoblast (CL0000515)
    Fold Change: 0.6246
    Cell Significance Index: 6.7900
  • Cell Name: cell in vitro (CL0001034)
    Fold Change: 0.6152
    Cell Significance Index: 335.9600
  • Cell Name: early pro-B cell (CL0002046)
    Fold Change: 0.6020
    Cell Significance Index: 38.8400
  • Cell Name: peg cell (CL4033014)
    Fold Change: 0.5861
    Cell Significance Index: 13.5400
  • Cell Name: fibroblast of dermis (CL0002551)
    Fold Change: 0.5700
    Cell Significance Index: 11.9300
  • Cell Name: placental villous trophoblast (CL2000060)
    Fold Change: 0.5610
    Cell Significance Index: 14.9800
  • Cell Name: cardiac muscle cell (CL0000746)
    Fold Change: 0.5419
    Cell Significance Index: 8.0000
  • Cell Name: tuft cell of colon (CL0009041)
    Fold Change: 0.5093
    Cell Significance Index: 459.8900
  • Cell Name: enterocyte of epithelium of small intestine (CL1000334)
    Fold Change: 0.5069
    Cell Significance Index: 14.6100
  • Cell Name: lactocyte (CL0002325)
    Fold Change: 0.5025
    Cell Significance Index: 64.9300
  • Cell Name: retinal rod cell (CL0000604)
    Fold Change: 0.4949
    Cell Significance Index: 5.9000
  • Cell Name: hair follicular keratinocyte (CL2000092)
    Fold Change: 0.4811
    Cell Significance Index: 212.7300
  • Cell Name: cardiac muscle myoblast (CL0000513)
    Fold Change: 0.4479
    Cell Significance Index: 34.3700
  • Cell Name: transit amplifying cell of colon (CL0009011)
    Fold Change: 0.4006
    Cell Significance Index: 12.8300
  • Cell Name: granulosa cell (CL0000501)
    Fold Change: 0.3704
    Cell Significance Index: 9.7400
  • Cell Name: neoplastic cell (CL0001063)
    Fold Change: 0.3612
    Cell Significance Index: 71.6800
  • Cell Name: enteroendocrine cell of colon (CL0009042)
    Fold Change: 0.3340
    Cell Significance Index: 63.5700
  • Cell Name: odontoblast (CL0000060)
    Fold Change: 0.3106
    Cell Significance Index: 39.8200
  • Cell Name: obsolete caudal ganglionic eminence derived GABAergic cortical interneuron (CL4023070)
    Fold Change: 0.2836
    Cell Significance Index: 101.7400
  • Cell Name: tonsil germinal center B cell (CL2000006)
    Fold Change: 0.2829
    Cell Significance Index: 33.3600
  • Cell Name: kidney cell (CL1000497)
    Fold Change: 0.2492
    Cell Significance Index: 1.9900
  • Cell Name: pancreatic acinar cell (CL0002064)
    Fold Change: 0.1936
    Cell Significance Index: 33.0600
  • Cell Name: lung endothelial cell (CL1001567)
    Fold Change: 0.1853
    Cell Significance Index: 9.6500
  • Cell Name: kidney loop of Henle cortical thick ascending limb epithelial cell (CL1001109)
    Fold Change: 0.1706
    Cell Significance Index: 125.0900
  • Cell Name: progenitor cell of mammary luminal epithelium (CL0009116)
    Fold Change: 0.1556
    Cell Significance Index: 11.6000
  • Cell Name: basal epithelial cell of tracheobronchial tree (CL0002329)
    Fold Change: 0.1270
    Cell Significance Index: 3.5500
  • Cell Name: GABAergic interneuron (CL0011005)
    Fold Change: 0.1177
    Cell Significance Index: 81.3800
  • Cell Name: small intestine goblet cell (CL1000495)
    Fold Change: 0.0720
    Cell Significance Index: 2.5300
  • Cell Name: intestinal crypt stem cell of small intestine (CL0009017)
    Fold Change: 0.0418
    Cell Significance Index: 0.8900
  • Cell Name: pigmented epithelial cell (CL0000529)
    Fold Change: 0.0380
    Cell Significance Index: 71.5200
  • Cell Name: colon goblet cell (CL0009039)
    Fold Change: 0.0250
    Cell Significance Index: 2.4800
  • Cell Name: kidney loop of Henle descending limb epithelial cell (CL1001021)
    Fold Change: 0.0093
    Cell Significance Index: 0.7400
  • Cell Name: non-pigmented ciliary epithelial cell (CL0002304)
    Fold Change: 0.0035
    Cell Significance Index: 2.2500
  • Cell Name: anterior lens cell (CL0002223)
    Fold Change: -0.0037
    Cell Significance Index: -6.8700
  • Cell Name: pulmonary alveolar epithelial cell (CL0000322)
    Fold Change: -0.0064
    Cell Significance Index: -4.8600
  • Cell Name: pancreatic PP cell (CL0002275)
    Fold Change: -0.0074
    Cell Significance Index: -4.5900
  • Cell Name: pancreatic A cell (CL0000171)
    Fold Change: -0.0088
    Cell Significance Index: -6.5000
  • Cell Name: lens epithelial cell (CL0002224)
    Fold Change: -0.0104
    Cell Significance Index: -16.0100
  • Cell Name: secondary lens fiber (CL0002225)
    Fold Change: -0.0286
    Cell Significance Index: -38.8200
  • Cell Name: ciliary muscle cell (CL1000443)
    Fold Change: -0.0362
    Cell Significance Index: -16.4500
  • Cell Name: abnormal cell (CL0001061)
    Fold Change: -0.0382
    Cell Significance Index: -3.9000
  • Cell Name: type B pancreatic cell (CL0000169)
    Fold Change: -0.0421
    Cell Significance Index: -23.7700
  • Cell Name: seromucus secreting cell (CL0000159)
    Fold Change: -0.0619
    Cell Significance Index: -1.2900
  • Cell Name: forebrain neuroblast (CL1000042)
    Fold Change: -0.0659
    Cell Significance Index: -4.0500
  • Cell Name: pancreatic D cell (CL0000173)
    Fold Change: -0.0864
    Cell Significance Index: -18.1900
  • Cell Name: pancreatic ductal cell (CL0002079)
    Fold Change: -0.1048
    Cell Significance Index: -12.0100
  • Cell Name: dopaminergic neuron (CL0000700)
    Fold Change: -0.1153
    Cell Significance Index: -33.1800
  • Cell Name: epithelial cell of stomach (CL0002178)
    Fold Change: -0.1206
    Cell Significance Index: -14.0500
  • Cell Name: pigmented ciliary epithelial cell (CL0002303)
    Fold Change: -0.1386
    Cell Significance Index: -20.1400
  • Cell Name: paneth cell of colon (CL0009009)
    Fold Change: -0.1462
    Cell Significance Index: -2.1900
  • Cell Name: enteroendocrine cell of small intestine (CL0009006)
    Fold Change: -0.1596
    Cell Significance Index: -3.9900
  • Cell Name: microfold cell of epithelium of small intestine (CL1000353)
    Fold Change: -0.1747
    Cell Significance Index: -12.0800
  • Cell Name: retinal progenitor cell (CL0002672)
    Fold Change: -0.1787
    Cell Significance Index: -10.0300
  • Cell Name: umbrella cell of urothelium (CL4030056)
    Fold Change: -0.2096
    Cell Significance Index: -1.9300
  • Cell Name: cone retinal bipolar cell (CL0000752)
    Fold Change: -0.2115
    Cell Significance Index: -1.6300
  • Cell Name: pro-T cell (CL0000827)
    Fold Change: -0.2161
    Cell Significance Index: -5.5200
  • Cell Name: pancreatic endocrine cell (CL0008024)
    Fold Change: -0.2174
    Cell Significance Index: -24.8200
  • Cell Name: CD4-positive, alpha-beta memory T cell, CD45RO-positive (CL0001204)
    Fold Change: -0.2217
    Cell Significance Index: -6.5100
  • Cell Name: paneth cell of epithelium of small intestine (CL1000343)
    Fold Change: -0.2340
    Cell Significance Index: -5.0700
  • Cell Name: hippocampal granule cell (CL0001033)
    Fold Change: -0.3325
    Cell Significance Index: -22.3600
  • Cell Name: smooth muscle cell of sphincter of pupil (CL0002243)
    Fold Change: -0.3513
    Cell Significance Index: -36.5800
  • Cell Name: fibroblast of mammary gland (CL0002555)
    Fold Change: -0.3593
    Cell Significance Index: -10.3000
  • Cell Name: mesenchymal cell (CL0008019)
    Fold Change: -0.3609
    Cell Significance Index: -6.0400
  • Cell Name: indirect pathway medium spiny neuron (CL4023029)
    Fold Change: -0.4072
    Cell Significance Index: -18.0100
  • Cell Name: cerebellar granule cell (CL0001031)
    Fold Change: -0.4073
    Cell Significance Index: -6.9800
  • Cell Name: type I muscle cell (CL0002211)
    Fold Change: -0.4543
    Cell Significance Index: -11.0900
  • Cell Name: direct pathway medium spiny neuron (CL4023026)
    Fold Change: -0.4670
    Cell Significance Index: -17.6900
  • Cell Name: intestinal tuft cell (CL0019032)
    Fold Change: -0.4998
    Cell Significance Index: -30.6400
  • Cell Name: cortical cell of adrenal gland (CL0002097)
    Fold Change: -0.5469
    Cell Significance Index: -14.6600
  • Cell Name: skeletal muscle fiber (CL0008002)
    Fold Change: -0.5765
    Cell Significance Index: -14.8200
  • Cell Name: intestinal epithelial cell (CL0002563)
    Fold Change: -0.6086
    Cell Significance Index: -6.3000

Cell ID: Standard Cell Ontology term used for mapping and comparing cells across experiments. Ensures consistency in analyzing cellular functions across tissues.
Fold Change: Represents the ratio of the current Cell Significance Index to the Cell Significance Index Threshold, indicating how much the gene expression has changed compared to a baseline.
Cell Significance Index: Reflects how strongly a gene is expressed in this specific cell.

Cell ID: Standard Cell Ontology term used for mapping and comparing cells across experiments. Ensures consistency in analyzing cellular functions across tissues.
Fold Change: Represents the ratio of the current Cell Significance Index to the Cell Significance Index Threshold, indicating how much the gene expression has changed compared to a baseline.
Cell Significance Index: Reflects how strongly a gene is expressed in this cell type. Calculated using techniques like effect size estimation and bootstrapping for reliability.

Cell ID: Standard Cell Ontology term used for mapping and comparing cells across experiments. Ensures consistency in analyzing cellular functions across tissues.
Fold Change: Represents the ratio of the current Cell Significance Index to the Cell Significance Index Threshold, indicating how much the gene expression has changed compared to a baseline.
Cell Significance Index: Reflects how strongly a gene is expressed in this cell type. Calculated using techniques like effect size estimation and bootstrapping for reliability.

Other Information

**Key Characteristics:** PDHA1 is a mitochondrial protein that belongs to the pyruvate dehydrogenase complex, which is a multi-enzyme complex that converts pyruvate to acetyl-CoA. It is composed of three subunits: E1, E2, and E3, with PDHA1 being the E1 subunit. PDHA1 is a highly conserved protein across species and is expressed in various tissues, including the brain, heart, and liver. It has a molecular weight of approximately 50 kDa and is composed of 395 amino acids. PDHA1 is a crucial component of the PDC and is essential for the proper functioning of the complex. **Pathways and Functions:** PDHA1 plays a central role in the regulation of cellular metabolism by catalyzing the conversion of pyruvate to acetyl-CoA. This reaction is a critical step in the transition from glycolysis to the citric acid cycle and is essential for the generation of energy in the form of ATP. PDHA1 is also involved in the regulation of the PDC through its interaction with other components of the complex, including the E2 and E3 subunits. Additionally, PDHA1 has been shown to play a role in the regulation of other metabolic pathways, including glycolysis and the citric acid cycle. **Regulation of PDHA1:** PDHA1 is regulated by various mechanisms, including phosphorylation, ubiquitination, and acetylation. Phosphorylation of PDHA1 by protein kinase A (PKA) inhibits its activity, while ubiquitination and acetylation promote its activity. These regulatory mechanisms are critical for the proper functioning of the PDC and the regulation of cellular metabolism. **Clinical Significance:** Dysregulation of PDHA1 has been implicated in various diseases, including cancer, neurodegenerative disorders, and metabolic disorders. Mutations in the PDHA1 gene have been associated with an increased risk of cancer, particularly in the brain and breast. Additionally, PDHA1 has been shown to play a role in the regulation of neurodegenerative disorders, such as Alzheimer's disease and Parkinson's disease. Furthermore, PDHA1 has been implicated in the regulation of metabolic disorders, including diabetes and obesity. **Signaling Pathways:** PDHA1 is involved in various signaling pathways, including the PI3K/AKT and MAPK/ERK pathways. These pathways play critical roles in the regulation of cellular metabolism and are involved in various diseases, including cancer and neurodegenerative disorders. **Conclusion:** In conclusion, PDHA1 is a crucial component of the pyruvate dehydrogenase complex and plays a central role in the regulation of cellular metabolism. Its dysregulation has been implicated in various diseases, including cancer, neurodegenerative disorders, and metabolic disorders. Further research is needed to fully understand the mechanisms of PDHA1 regulation and its clinical significance.

Genular Protein ID: 4228403840

Symbol: ODPA_HUMAN

Name: Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial

UniProtKB Accession Codes:

Database IDs:

Citations:

PubMed ID: 2227443

Title: Characterization and nucleotide sequence of the gene encoding the human pyruvate dehydrogenase alpha-subunit.

PubMed ID: 2227443

DOI: 10.1016/0378-1119(90)90241-i

PubMed ID: 2748588

Title: Characterization of cDNAs encoding human pyruvate dehydrogenase alpha subunit.

PubMed ID: 2748588

DOI: 10.1073/pnas.86.14.5330

PubMed ID: 3034892

Title: The human pyruvate dehydrogenase complex. Isolation of cDNA clones for the E1 alpha subunit, sequence analysis, and characterization of the mRNA.

PubMed ID: 3034892

DOI: 10.1016/s0021-9258(18)48250-6

PubMed ID: 2745444

Title: Structural organization of the gene for the E1 alpha subunit of the human pyruvate dehydrogenase complex.

PubMed ID: 2745444

DOI: 10.1016/s0021-9258(18)63857-8

PubMed ID: 2828359

Title: Isolation of a full-length complementary DNA coding for human E1 alpha subunit of the pyruvate dehydrogenase complex.

PubMed ID: 2828359

DOI: 10.1016/s0021-9258(19)77975-7

PubMed ID: 3422424

Title: Cloning and sequencing of cDNAs encoding alpha and beta subunits of human pyruvate dehydrogenase.

PubMed ID: 3422424

DOI: 10.1073/pnas.85.1.41

PubMed ID: 14702039

Title: Complete sequencing and characterization of 21,243 full-length human cDNAs.

PubMed ID: 14702039

DOI: 10.1038/ng1285

PubMed ID: 15772651

Title: The DNA sequence of the human X chromosome.

PubMed ID: 15772651

DOI: 10.1038/nature03440

PubMed ID: 15489334

Title: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).

PubMed ID: 15489334

DOI: 10.1101/gr.2596504

PubMed ID: 23146587

Title: Experimental determination of organelle targeting-peptide cleavage sites using transient expression of green fluorescent protein translational fusions.

PubMed ID: 23146587

DOI: 10.1016/j.ab.2012.10.040

PubMed ID: 10077682

Title: X chromosome evidence for ancient human histories.

PubMed ID: 10077682

DOI: 10.1073/pnas.96.6.3320

PubMed ID: 1338114

Title: Mutation of E1 alpha gene in a female patient with pyruvate dehydrogenase deficiency due to rapid degradation of E1 protein.

PubMed ID: 1338114

DOI: 10.1007/bf01800220

PubMed ID: 7782287

Title: Mutagenesis studies of the phosphorylation sites of recombinant human pyruvate dehydrogenase. Site-specific regulation.

PubMed ID: 7782287

DOI: 10.1074/jbc.270.24.14297

PubMed ID: 11486000

Title: Site specificity of four pyruvate dehydrogenase kinase isoenzymes toward the three phosphorylation sites of human pyruvate dehydrogenase.

PubMed ID: 11486000

DOI: 10.1074/jbc.m103069200

PubMed ID: 14638692

Title: Organization of the cores of the mammalian pyruvate dehydrogenase complex formed by E2 and E2 plus the E3-binding protein and their capacities to bind the E1 and E3 components.

PubMed ID: 14638692

DOI: 10.1074/jbc.m308172200

PubMed ID: 17081983

Title: Global, in vivo, and site-specific phosphorylation dynamics in signaling networks.

PubMed ID: 17081983

DOI: 10.1016/j.cell.2006.09.026

PubMed ID: 18088087

Title: Phosphoproteome of resting human platelets.

PubMed ID: 18088087

DOI: 10.1021/pr0704130

PubMed ID: 18691976

Title: Kinase-selective enrichment enables quantitative phosphoproteomics of the kinome across the cell cycle.

PubMed ID: 18691976

DOI: 10.1016/j.molcel.2008.07.007

PubMed ID: 18669648

Title: A quantitative atlas of mitotic phosphorylation.

PubMed ID: 18669648

DOI: 10.1073/pnas.0805139105

PubMed ID: 19413330

Title: Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach.

PubMed ID: 19413330

DOI: 10.1021/ac9004309

PubMed ID: 19608861

Title: Lysine acetylation targets protein complexes and co-regulates major cellular functions.

PubMed ID: 19608861

DOI: 10.1126/science.1175371

PubMed ID: 20068231

Title: Quantitative phosphoproteomics reveals widespread full phosphorylation site occupancy during mitosis.

PubMed ID: 20068231

DOI: 10.1126/scisignal.2000475

PubMed ID: 21269460

Title: Initial characterization of the human central proteome.

PubMed ID: 21269460

DOI: 10.1186/1752-0509-5-17

PubMed ID: 21406692

Title: System-wide temporal characterization of the proteome and phosphoproteome of human embryonic stem cell differentiation.

PubMed ID: 21406692

DOI: 10.1126/scisignal.2001570

PubMed ID: 23186163

Title: Toward a comprehensive characterization of a human cancer cell phosphoproteome.

PubMed ID: 23186163

DOI: 10.1021/pr300630k

PubMed ID: 24275569

Title: An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.

PubMed ID: 24275569

DOI: 10.1016/j.jprot.2013.11.014

PubMed ID: 25944712

Title: N-terminome analysis of the human mitochondrial proteome.

PubMed ID: 25944712

DOI: 10.1002/pmic.201400617

PubMed ID: 12651851

Title: Structural basis for flip-flop action of thiamin pyrophosphate-dependent enzymes revealed by human pyruvate dehydrogenase.

PubMed ID: 12651851

DOI: 10.1074/jbc.m300339200

PubMed ID: 17474719

Title: Phosphorylation of serine 264 impedes active site accessibility in the E1 component of the human pyruvate dehydrogenase multienzyme complex.

PubMed ID: 17474719

DOI: 10.1021/bi700083z

PubMed ID: 19081061

Title: Structural basis for inactivation of the human pyruvate dehydrogenase complex by phosphorylation: role of disordered phosphorylation loops.

PubMed ID: 19081061

DOI: 10.1016/j.str.2008.10.010

PubMed ID: 29970614

Title: Pyruvate dehydrogenase complex deficiency is linked to regulatory loop disorder in the alphaV138M variant of human pyruvate dehydrogenase.

PubMed ID: 29970614

DOI: 10.1074/jbc.ra118.003996

PubMed ID: 1301207

Title: Mutations and polymorphisms in the pyruvate dehydrogenase E1 alpha gene.

PubMed ID: 1301207

DOI: 10.1002/humu.1380010203

PubMed ID: 1909401

Title: Characterization of the mutations in three patients with pyruvate dehydrogenase E1 alpha deficiency.

PubMed ID: 1909401

DOI: 10.1007/bf01800586

PubMed ID: 1551669

Title: Pyruvate dehydrogenase (PDH) deficiency caused by a 21-base pair insertion mutation in the E1 alpha subunit.

PubMed ID: 1551669

DOI: 10.1007/bf02265291

PubMed ID: 1293379

Title: X-linked pyruvate dehydrogenase E1 alpha subunit deficiency in heterozygous females: variable manifestation of the same mutation.

PubMed ID: 1293379

DOI: 10.1007/bf01800219

PubMed ID: 8498846

Title: Molecular genetic characterization of an X-linked form of Leigh's syndrome.

PubMed ID: 8498846

DOI: 10.1002/ana.410330616

PubMed ID: 8504306

Title: Mutations in the X-linked E1 alpha subunit of pyruvate dehydrogenase leading to deficiency of the pyruvate dehydrogenase complex.

PubMed ID: 8504306

DOI: 10.1093/hmg/2.4.449

PubMed ID: 8032855

Title: Pyruvate dehydrogenase deficiency. Clinical presentation and molecular genetic characterization of five new patients.

PubMed ID: 8032855

DOI: 10.1093/brain/117.3.435

PubMed ID: 7545958

Title: Pyruvate dehydrogenase deficiency caused by a 33 base pair duplication in the PDH E1 alpha subunit.

PubMed ID: 7545958

DOI: 10.1093/hmg/3.6.1021

PubMed ID: 8199595

Title: Pyruvate dehydrogenase deficiency in a male caused by a point mutation (F205L) in the E1 alpha subunit.

PubMed ID: 8199595

DOI: 10.1002/humu.1380030210

PubMed ID: 7967473

Title: Characterization of a point mutation in the pyruvate dehydrogenase E1 alpha gene from two boys with primary lactic acidaemia.

PubMed ID: 7967473

DOI: 10.1007/bf00711616

PubMed ID: 7887409

Title: Mutations in the X-linked E1 alpha subunit of pyruvate dehydrogenase: exon skipping, insertion of duplicate sequence, and missense mutations leading to the deficiency of the pyruvate dehydrogenase complex.

PubMed ID: 7887409

PubMed ID: 7573035

Title: An amino acid substitution in the pyruvate dehydrogenase E1 alpha gene, affecting mitochondrial import of the precursor protein.

PubMed ID: 7573035

PubMed ID: 7757088

Title: Pyruvate dehydrogenase complex deficiency due to a point mutation (P188L) within the thiamine pyrophosphate binding loop of the E1 alpha subunit.

PubMed ID: 7757088

DOI: 10.1093/hmg/4.2.315

PubMed ID: 8664900

Title: Mutation analysis of the pyruvate dehydrogenase E1 alpha gene in eight patients with a pyruvate dehydrogenase complex deficiency.

PubMed ID: 8664900

DOI: 10.1002/(sici)1098-1004(1996)7:1<46::aid-humu6>3.0.co;2-n

PubMed ID: 8844217

Title: Three new mutations of the pyruvate dehydrogenase alpha subunit: a point mutation (M181V), 3 bp deletion (-R282), and 16 bp insertion/frameshift (K358SVS-->TVDQS).

PubMed ID: 8844217

DOI: 10.1002/(sici)1098-1004(1996)8:2<180::aid-humu11>3.0.co;2-z

PubMed ID: 9266390

Title: Biochemical and molecular analysis of an X-linked case of Leigh syndrome associated with thiamin-responsive pyruvate dehydrogenase deficiency.

PubMed ID: 9266390

DOI: 10.1023/a:1005305614374

PubMed ID: 9671272

Title: Arginine 302 mutations in the pyruvate dehydrogenase E1alpha subunit gene: identification of further patients and in vitro demonstration of pathogenicity.

PubMed ID: 9671272

DOI: 10.1002/(sici)1098-1004(1998)12:2<114::aid-humu6>3.0.co;2-#

PubMed ID: 23033978

Title: Diagnostic exome sequencing in persons with severe intellectual disability.

PubMed ID: 23033978

DOI: 10.1056/nejmoa1206524

PubMed ID: 27864847

Title: Diagnostic targeted resequencing in 349 patients with drug-resistant pediatric epilepsies identifies causative mutations in 30 different genes.

PubMed ID: 27864847

DOI: 10.1002/humu.23149

Sequence Information:

  • Length: 390
  • Mass: 43296
  • Checksum: 4D685BBE44A92D4B
  • Sequence:
  • MRKMLAAVSR VLSGASQKPA SRVLVASRNF ANDATFEIKK CDLHRLEEGP PVTTVLTRED 
    GLKYYRMMQT VRRMELKADQ LYKQKIIRGF CHLCDGQEAC CVGLEAGINP TDHLITAYRA 
    HGFTFTRGLS VREILAELTG RKGGCAKGKG GSMHMYAKNF YGGNGIVGAQ VPLGAGIALA 
    CKYNGKDEVC LTLYGDGAAN QGQIFEAYNM AALWKLPCIF ICENNRYGMG TSVERAAAST 
    DYYKRGDFIP GLRVDGMDIL CVREATRFAA AYCRSGKGPI LMELQTYRYH GHSMSDPGVS 
    YRTREEIQEV RSKSDPIMLL KDRMVNSNLA SVEELKEIDV EVRKEIEDAA QFATADPEPP 
    LEELGYHIYS SDPPFEVRGA NQWIKFKSVS

Database document:

This is a preview of the gene's schema. Only a few entries are kept for 'singleCellExpressions,' 'mRNAExpressions,' and other large data arrays for visualization purposes. You can zoom in with the mouse wheel for a closer view, and the text will adjust automatically if necessary. For the full schema, download it here.