MONDO:0009502 |
DLAT |
P10515 |
2.3.1.12
|
R-HSA-204174
R-HSA-204174
Reactome Name: Regulation of pyruvate dehydrogenase (PDH) complex
See in Reactome
R-HSA-389661
R-HSA-389661
Reactome Name: Glyoxylate metabolism and glycine degradation
See in Reactome
R-HSA-70268
R-HSA-5362517
|
R-HSA-71406
R-HSA-71406
Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle
See in Reactome
R-HSA-71291
R-HSA-71291
Reactome Name: Metabolism of amino acids and derivatives
See in Reactome
R-HSA-9006931
R-HSA-9006931
Reactome Name: Signaling by Nuclear Receptors
See in Reactome
R-HSA-1430728
R-HSA-1428517
R-HSA-1428517
Reactome Name: The citric acid (TCA) cycle and respiratory electron transport
See in Reactome
R-HSA-162582
|
Metabolism
Signal Transduction
|
R-HSA-203946
(
R-HSA-204174;
R-HSA-5362517;
R-HSA-70268
)
R-HSA-203946
Reaction Name: PDK isoforms phosphorylate lipo-PDH
See in Reactome
R-HSA-204169
(
R-HSA-204174;
R-HSA-70268
)
R-HSA-204169
Reaction Name: PDP dephosphorylates p-lipo-PDH
See in Reactome
R-HSA-6792572
(
R-HSA-389661
)
R-HSA-6792572
Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs
See in Reactome
R-HSA-71397
(
R-HSA-70268
)
R-HSA-71397
Reaction Name: lipo-PDH decarboxylates PYR to Ac-CoA
See in Reactome
|
MONDO:0009529 |
DLD |
P09622 |
1.8.1.4
|
R-HSA-71403
R-HSA-204174
R-HSA-204174
Reactome Name: Regulation of pyruvate dehydrogenase (PDH) complex
See in Reactome
R-HSA-70895
R-HSA-70895
Reactome Name: Branched-chain amino acid catabolism
See in Reactome
R-HSA-70268
R-HSA-71064
R-HSA-6783984
R-HSA-389661
R-HSA-389661
Reactome Name: Glyoxylate metabolism and glycine degradation
See in Reactome
R-HSA-5362517
|
R-HSA-71406
R-HSA-71406
Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle
See in Reactome
R-HSA-71291
R-HSA-71291
Reactome Name: Metabolism of amino acids and derivatives
See in Reactome
R-HSA-9006931
R-HSA-9006931
Reactome Name: Signaling by Nuclear Receptors
See in Reactome
R-HSA-1430728
R-HSA-1428517
R-HSA-1428517
Reactome Name: The citric acid (TCA) cycle and respiratory electron transport
See in Reactome
R-HSA-162582
|
Metabolism
Signal Transduction
|
R-HSA-203946
(
R-HSA-204174;
R-HSA-5362517;
R-HSA-70268
)
R-HSA-203946
Reaction Name: PDK isoforms phosphorylate lipo-PDH
See in Reactome
R-HSA-204169
(
R-HSA-204174;
R-HSA-70268
)
R-HSA-204169
Reaction Name: PDP dephosphorylates p-lipo-PDH
See in Reactome
R-HSA-5693148
(
R-HSA-70895
)
R-HSA-5693153
(
R-HSA-70895
)
R-HSA-5693153
Reaction Name: PPM1K dephosphorylates p-BCKDH
See in Reactome
R-HSA-5694018
(
R-HSA-389661;
R-HSA-6783984
)
R-HSA-5694018
Reaction Name: DLD dimer:2xFAD oxidises GCSH:DHLL to GCSH:lipoate
See in Reactome
R-HSA-6792572
(
R-HSA-389661
)
R-HSA-6792572
Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs
See in Reactome
R-HSA-70713
(
R-HSA-70895
)
R-HSA-70713
Reaction Name: BCKDH transfers CoA group from CoA-SH to BCAAs
See in Reactome
R-HSA-71037
(
R-HSA-71064
)
R-HSA-71037
Reaction Name: alpha-ketoadipate + CoASH + NAD+ => glutaryl-CoA + CO2 + NADH + H+
See in Reactome
R-HSA-71397
(
R-HSA-70268
)
R-HSA-71397
Reaction Name: lipo-PDH decarboxylates PYR to Ac-CoA
See in Reactome
R-HSA-71401
(
R-HSA-71403
)
R-HSA-71401
Reaction Name: alpha-ketoglutarate + CoASH + NAD+ => succinyl-CoA + CO2 + NADH + H+
See in Reactome
|
MONDO:0009776 |
MONDO:0009776
Disease Name: spermatogenic failure 1
OMIM id: OMIM:258150
All associated enzymes in DAR:
PDHA2
. The disease is also associated to other non-enzyme proteins (not in DAR).
PDHA2 |
P29803 |
1.2.4.1
|
R-HSA-204174
R-HSA-204174
Reactome Name: Regulation of pyruvate dehydrogenase (PDH) complex
See in Reactome
R-HSA-389661
R-HSA-389661
Reactome Name: Glyoxylate metabolism and glycine degradation
See in Reactome
R-HSA-70268
R-HSA-5362517
|
R-HSA-71406
R-HSA-71406
Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle
See in Reactome
R-HSA-71291
R-HSA-71291
Reactome Name: Metabolism of amino acids and derivatives
See in Reactome
R-HSA-9006931
R-HSA-9006931
Reactome Name: Signaling by Nuclear Receptors
See in Reactome
R-HSA-1430728
R-HSA-1428517
R-HSA-1428517
Reactome Name: The citric acid (TCA) cycle and respiratory electron transport
See in Reactome
R-HSA-162582
|
Metabolism
Signal Transduction
|
R-HSA-203946
(
R-HSA-204174;
R-HSA-5362517;
R-HSA-70268
)
R-HSA-203946
Reaction Name: PDK isoforms phosphorylate lipo-PDH
See in Reactome
R-HSA-204169
(
R-HSA-204174;
R-HSA-70268
)
R-HSA-204169
Reaction Name: PDP dephosphorylates p-lipo-PDH
See in Reactome
R-HSA-6792572
(
R-HSA-389661
)
R-HSA-6792572
Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs
See in Reactome
R-HSA-71397
(
R-HSA-70268
)
R-HSA-71397
Reaction Name: lipo-PDH decarboxylates PYR to Ac-CoA
See in Reactome
|
MONDO:0010479 |
PDK3 |
Q15120 |
2.7.11.2
|
R-HSA-204174
R-HSA-204174
Reactome Name: Regulation of pyruvate dehydrogenase (PDH) complex
See in Reactome
R-HSA-5362517
|
R-HSA-71406
R-HSA-71406
Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle
See in Reactome
R-HSA-1428517
R-HSA-1428517
Reactome Name: The citric acid (TCA) cycle and respiratory electron transport
See in Reactome
R-HSA-9006931
R-HSA-9006931
Reactome Name: Signaling by Nuclear Receptors
See in Reactome
R-HSA-70268
R-HSA-1430728
R-HSA-162582
|
Metabolism
Signal Transduction
|
R-HSA-203946
(
R-HSA-204174;
R-HSA-5362517;
R-HSA-70268
)
R-HSA-203946
Reaction Name: PDK isoforms phosphorylate lipo-PDH
See in Reactome
|
MONDO:0010717 |
PDHA1 |
P08559 |
1.2.4.1
|
R-HSA-204174
R-HSA-204174
Reactome Name: Regulation of pyruvate dehydrogenase (PDH) complex
See in Reactome
R-HSA-389661
R-HSA-389661
Reactome Name: Glyoxylate metabolism and glycine degradation
See in Reactome
R-HSA-70268
R-HSA-5362517
|
R-HSA-71406
R-HSA-71406
Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle
See in Reactome
R-HSA-71291
R-HSA-71291
Reactome Name: Metabolism of amino acids and derivatives
See in Reactome
R-HSA-9006931
R-HSA-9006931
Reactome Name: Signaling by Nuclear Receptors
See in Reactome
R-HSA-1430728
R-HSA-1428517
R-HSA-1428517
Reactome Name: The citric acid (TCA) cycle and respiratory electron transport
See in Reactome
R-HSA-162582
|
Metabolism
Signal Transduction
|
R-HSA-203946
(
R-HSA-204174;
R-HSA-5362517;
R-HSA-70268
)
R-HSA-203946
Reaction Name: PDK isoforms phosphorylate lipo-PDH
See in Reactome
R-HSA-204169
(
R-HSA-204174;
R-HSA-70268
)
R-HSA-204169
Reaction Name: PDP dephosphorylates p-lipo-PDH
See in Reactome
R-HSA-6792572
(
R-HSA-389661
)
R-HSA-6792572
Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs
See in Reactome
R-HSA-71397
(
R-HSA-70268
)
R-HSA-71397
Reaction Name: lipo-PDH decarboxylates PYR to Ac-CoA
See in Reactome
|
MONDO:0012120 |
PDP1 |
Q9P0J1 |
3.1.3.43
|
R-HSA-204174
R-HSA-204174
Reactome Name: Regulation of pyruvate dehydrogenase (PDH) complex
See in Reactome
|
R-HSA-1428517
R-HSA-1428517
Reactome Name: The citric acid (TCA) cycle and respiratory electron transport
See in Reactome
R-HSA-70268
R-HSA-71406
R-HSA-71406
Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle
See in Reactome
R-HSA-1430728
|
Metabolism
|
R-HSA-204169
(
R-HSA-204174;
R-HSA-70268
)
R-HSA-204169
Reaction Name: PDP dephosphorylates p-lipo-PDH
See in Reactome
|
MONDO:0013580 |
PDHB |
P11177 |
1.2.4.1
|
R-HSA-204174
R-HSA-204174
Reactome Name: Regulation of pyruvate dehydrogenase (PDH) complex
See in Reactome
R-HSA-389661
R-HSA-389661
Reactome Name: Glyoxylate metabolism and glycine degradation
See in Reactome
R-HSA-70268
R-HSA-5362517
|
R-HSA-71406
R-HSA-71406
Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle
See in Reactome
R-HSA-71291
R-HSA-71291
Reactome Name: Metabolism of amino acids and derivatives
See in Reactome
R-HSA-9006931
R-HSA-9006931
Reactome Name: Signaling by Nuclear Receptors
See in Reactome
R-HSA-1430728
R-HSA-1428517
R-HSA-1428517
Reactome Name: The citric acid (TCA) cycle and respiratory electron transport
See in Reactome
R-HSA-162582
|
Metabolism
Signal Transduction
|
R-HSA-203946
(
R-HSA-204174;
R-HSA-5362517;
R-HSA-70268
)
R-HSA-203946
Reaction Name: PDK isoforms phosphorylate lipo-PDH
See in Reactome
R-HSA-204169
(
R-HSA-204174;
R-HSA-70268
)
R-HSA-204169
Reaction Name: PDP dephosphorylates p-lipo-PDH
See in Reactome
R-HSA-6792572
(
R-HSA-389661
)
R-HSA-6792572
Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs
See in Reactome
R-HSA-71397
(
R-HSA-70268
)
R-HSA-71397
Reaction Name: lipo-PDH decarboxylates PYR to Ac-CoA
See in Reactome
|
MONDO:0016815 |
PDHA1 |
P08559 |
1.2.4.1
|
R-HSA-204174
R-HSA-204174
Reactome Name: Regulation of pyruvate dehydrogenase (PDH) complex
See in Reactome
R-HSA-389661
R-HSA-389661
Reactome Name: Glyoxylate metabolism and glycine degradation
See in Reactome
R-HSA-70268
R-HSA-5362517
|
R-HSA-71406
R-HSA-71406
Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle
See in Reactome
R-HSA-71291
R-HSA-71291
Reactome Name: Metabolism of amino acids and derivatives
See in Reactome
R-HSA-9006931
R-HSA-9006931
Reactome Name: Signaling by Nuclear Receptors
See in Reactome
R-HSA-1430728
R-HSA-1428517
R-HSA-1428517
Reactome Name: The citric acid (TCA) cycle and respiratory electron transport
See in Reactome
R-HSA-162582
|
Metabolism
Signal Transduction
|
R-HSA-203946
(
R-HSA-204174;
R-HSA-5362517;
R-HSA-70268
)
R-HSA-203946
Reaction Name: PDK isoforms phosphorylate lipo-PDH
See in Reactome
R-HSA-204169
(
R-HSA-204174;
R-HSA-70268
)
R-HSA-204169
Reaction Name: PDP dephosphorylates p-lipo-PDH
See in Reactome
R-HSA-6792572
(
R-HSA-389661
)
R-HSA-6792572
Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs
See in Reactome
R-HSA-71397
(
R-HSA-70268
)
R-HSA-71397
Reaction Name: lipo-PDH decarboxylates PYR to Ac-CoA
See in Reactome
|
MONDO:0060527 |
GSTZ1 |
O43708 |
5.2.1.2
2.5.1.18
|
R-HSA-204174
R-HSA-204174
Reactome Name: Regulation of pyruvate dehydrogenase (PDH) complex
See in Reactome
R-HSA-8963684
R-HSA-156590
|
R-HSA-71406
R-HSA-71406
Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle
See in Reactome
R-HSA-211859
R-HSA-71291
R-HSA-71291
Reactome Name: Metabolism of amino acids and derivatives
See in Reactome
R-HSA-156580
R-HSA-156580
Reactome Name: Phase II - Conjugation of compounds
See in Reactome
R-HSA-70268
R-HSA-1430728
R-HSA-1428517
R-HSA-1428517
Reactome Name: The citric acid (TCA) cycle and respiratory electron transport
See in Reactome
R-HSA-8963691
R-HSA-8963691
Reactome Name: Phenylalanine and tyrosine metabolism
See in Reactome
|
Metabolism
|
R-HSA-176054
(
R-HSA-156590
)
R-HSA-176054
Reaction Name: GST dimers conjugate GSH with cytosolic substrates
See in Reactome
R-HSA-71173
(
R-HSA-8963684
)
R-HSA-71173
Reaction Name: maleylacetoacetate => fumarylacetoacetate
See in Reactome
R-HSA-9011595
(
R-HSA-204174;
R-HSA-70268
)
R-HSA-9011595
Reaction Name: GSTZ1 dimer dehalogenates DCA to glyoxylate
See in Reactome
|