KEGG   ORTHOLOGY: K07748Help
Entry
K07748                      KO                                     

Name
NSDHL, ERG26
Definition
sterol-4alpha-carboxylate 3-dehydrogenase (decarboxylating) [EC:1.1.1.170]
Pathway
ko00100  Steroid biosynthesis
ko01100  Metabolic pathways
ko01130  Biosynthesis of antibiotics
Module
M00101  Cholesterol biosynthesis, squalene 2,3-epoxide => cholesterol
Disease
H00496  Congenital hemidysplasia with ichthyosiform nevus and limb defects (CHILD)
H01917  CK syndrome
Brite
KEGG Orthology (KO) [BR:ko00001]
 09100 Metabolism
  09103 Lipid metabolism
   00100 Steroid biosynthesis
    K07748  NSDHL, ERG26; sterol-4alpha-carboxylate 3-dehydrogenase (decarboxylating)
Enzymes [BR:ko01000]
 1. Oxidoreductases
  1.1  Acting on the CH-OH group of donors
   1.1.1  With NAD+ or NADP+ as acceptor
    1.1.1.170  3beta-hydroxysteroid-4alpha-carboxylate 3-dehydrogenase (decarboxylating)
     K07748  NSDHL, ERG26; sterol-4alpha-carboxylate 3-dehydrogenase (decarboxylating)
BRITE hierarchy
Other DBs
RN: R07494 R12404
COG: COG0451
GO: 0047012
Genes
HSA: 50814(NSDHL)
PTR: 465960(NSDHL)
PPS: 100975917(NSDHL)
GGO: 101144867(NSDHL)
PON: 100172372(NSDHL)
NLE: 100596441(NSDHL)
MCC: 693333(NSDHL)
MCF: 102137627(NSDHL)
CSAB: 103232767(NSDHL)
RRO: 104654022(NSDHL)
RBB: 108513868(NSDHL)
CJC: 100398582(NSDHL)
SBQ: 101027738(NSDHL)
MMU: 18194(Nsdhl)
MCAL: 110287212(Nsdhl)
MPAH: 110313833(Nsdhl)
RNO: 309262(Nsdhl)
MUN: 110540271(Nsdhl)
CGE: 100764429(Nsdhl) 100768776
NGI: 103731179(Nsdhl)
HGL: 101716507(Nsdhl)
CCAN: 109695438(Nsdhl)
OCU: 100351296(NSDHL)
TUP: 102500582(NSDHL)
CFA: 481079(NSDHL)
VVP: 112912365(NSDHL)
AML: 100466780(NSDHL)
UMR: 103674163(NSDHL)
UAH: 113246811(NSDHL)
ORO: 101372464(NSDHL)
ELK: 111139213
FCA: 101096551(NSDHL)
PTG: 102959228(NSDHL)
PPAD: 109260189(NSDHL)
AJU: 106971243(NSDHL)
BTA: 616694(NSDHL)
BOM: 102276234(NSDHL)
BIU: 109555394(NSDHL)
BBUB: 102398147(NSDHL)
CHX: 102173170(NSDHL)
OAS: 101112792(NSDHL)
SSC: 100157894(NSDHL)
CFR: 102516550(NSDHL)
CDK: 105104051(NSDHL)
BACU: 103020703(NSDHL)
LVE: 103087463(NSDHL)
OOR: 101278134(NSDHL)
DLE: 111170601(NSDHL)
PCAD: 102989952(NSDHL)
ECB: 100063648(NSDHL)
EPZ: 103543383(NSDHL)
EAI: 106834275(NSDHL)
MYB: 102257708(NSDHL)
MYD: 102771848(NSDHL)
MNA: 107537970(NSDHL)
HAI: 109394344(NSDHL)
DRO: 112296338(NSDHL)
PALE: 102879960(NSDHL)
RAY: 107520609(NSDHL)
MJV: 108402575(NSDHL)
LAV: 100671133(NSDHL)
TMU: 101357526
MDO: 100026414(NSDHL)
SHR: 100914940(NSDHL)
PCW: 110199978(NSDHL)
OAA: 103171093(NSDHL)
GGA: 422302(NSDHL)
MGP: 100544971(NSDHL)
CJO: 107312870(NSDHL)
NMEL: 110402996(NSDHL)
APLA: 101790127(NSDHL)
ACYG: 106047509(NSDHL)
TGU: 100190543(NSDHL)
LSR: 110477979(NSDHL)
SCAN: 103816322(NSDHL)
GFR: 102041531(NSDHL)
FAB: 101814664(NSDHL)
PHI: 102100867(NSDHL)
PMAJ: 107204042(NSDHL)
CCAE: 111929260(NSDHL)
CCW: 104691595(NSDHL)
ETL: 114062107(NSDHL)
FPG: 101921503(NSDHL)
FCH: 102048326(NSDHL)
CLV: 102098029(NSDHL)
EGZ: 104127975(NSDHL)
NNI: 104021094(NSDHL)
ACUN: 113480109(NSDHL)
PADL: 103925887(NSDHL)
AAM: 106489838(NSDHL)
ASN: 102383140(NSDHL)
AMJ: 102562140(NSDHL)
PSS: 102447619(NSDHL)
CMY: 102945577(NSDHL)
CPIC: 101937255(NSDHL)
ACS: 100562829(nsdhl)
PVT: 110088403(NSDHL)
PBI: 103061373(NSDHL)
PMUR: 107293162(NSDHL)
TSR: 106555925(NSDHL)
PMUA: 114589018(NSDHL)
GJA: 107118212(NSDHL)
XLA: 108700163(nsdhl.S) 496236(nsdhl.L)
XTR: 550044(nsdhl)
NPR: 108795343(NSDHL)
DRE: 550369(nsdhl)
SRX: 107733445(nsdhl)
SANH: 107663101(nsdhl)
SGH: 107554660(nsdhl)
IPU: 108268902(nsdhl)
PHYP: 113542904(nsdhl)
AMEX: 103027604(nsdhl)
EEE: 113576547(nsdhl)
TRU: 101061359(nsdhl)
LCO: 104923201(nsdhl)
NCC: 104948435(nsdhl)
MZE: 101480912(nsdhl)
ONL: 100712373(nsdhl)
OLA: 101171010(nsdhl)
XMA: 102221009(nsdhl)
XCO: 114139190(nsdhl)
PRET: 103471168(nsdhl)
CVG: 107089032(nsdhl)
NFU: 107395696(nsdhl)
KMR: 108242032(nsdhl)
ALIM: 106515962(nsdhl)
AOCE: 111565582(nsdhl)
CSEM: 103391149(nsdhl)
POV: 109624038(nsdhl)
LCF: 108882885(nsdhl)
SDU: 111226496(nsdhl)
SLAL: 111652007(nsdhl)
HCQ: 109515829(nsdhl)
BPEC: 110163595(nsdhl)
MALB: 109968402(nsdhl)
ELS: 105025751(nsdhl)
SFM: 108925361(nsdhl)
PKI: 111843704(nsdhl)
LCM: 102355835(NSDHL)
CMK: 103177331(nsdhl)
RTP: 109917662(nsdhl)
SPU: 577670
APLC: 110983741
SKO: 100367277
PCAN: 112560719
LAK: 106150518
AQU: 100641976
CRE: CHLREDRAFT_58501(BHSD1)
MNG: MNEG_5826
APRO: F751_0534
SCE: YGL001C(ERG26)
ERC: Ecym_6178
KMX: KLMA_40026(ERG26)
NCS: NCAS_0A05210(NCAS0A05210)
NDI: NDAI_0K02600(NDAI0K02600)
TPF: TPHA_0K01550(TPHA0K01550)
TBL: TBLA_0E01630(TBLA0E01630)
TDL: TDEL_0F02190(TDEL0F02190)
KAF: KAFR_0F03320(KAFR0F03320)
PIC: PICST_71224(ERG26)
CAL: CAALFM_C406270CA(ERG26)
CDU: CD36_45790(ERG26)
SLB: AWJ20_2805(ERG26)
NCR: NCU02693
NTE: NEUTE1DRAFT119017(NEUTE1DRAFT_119017)
CMT: CCM_07037
MBE: MBM_00113
ANG: ANI_1_470134(An15g03090)
PBL: PAAG_11343(PAAG_01699)
PTE: PTT_14047
CNE: CNJ00460
CNB: CNBJ3060
ABP: AGABI1DRAFT113991(AGABI1DRAFT_113991)
ABV: AGABI2DRAFT193828(AGABI2DRAFT_193828)
MGL: MGL_3391
MRT: MRET_1855
SPAR: SPRG_01499
 » show all
TaxonomyKoalaUniProt
Reference
PMID:7430141
  Authors
Brady DR, Crowder RD, Hayes WJ
  Title
Mixed function oxidases in sterol metabolism. Source of reducing equivalents.
  Journal
J Biol Chem 255:10624-9 (1980)
Reference
PMID:9811880
  Authors
Gachotte D, Barbuch R, Gaylor J, Nickel E, Bard M
  Title
Characterization of the Saccharomyces cerevisiae ERG26 gene encoding the C-3 sterol dehydrogenase (C-4 decarboxylase) involved in sterol biosynthesis.
  Journal
Proc Natl Acad Sci U S A 95:13794-9 (1998)
DOI:10.1073/pnas.95.23.13794
  Sequence
[sce:YGL001C]

KEGG   ENZYME: 1.1.1.170Help
Entry
EC 1.1.1.170                Enzyme                                 

Name
3beta-hydroxysteroid-4alpha-carboxylate 3-dehydrogenase (decarboxylating);
3beta-hydroxy-4beta-methylcholestenecarboxylate 3-dehydrogenase (decarboxylating);
3beta-hydroxy-4beta-methylcholestenoate dehydrogenase;
sterol 4alpha-carboxylic decarboxylase;
sterol-4alpha-carboxylate 3-dehydrogenase (decarboxylating) (ambiguous);
ERG26 (gene name);
NSDHL (gene name)
Class
Oxidoreductases;
Acting on the CH-OH group of donors;
With NAD+ or NADP+ as acceptor
BRITE hierarchy
Sysname
3beta-hydroxysteroid-4alpha-carboxylate:NAD(P)+ 3-oxidoreductase (decarboxylating)
Reaction(IUBMB)
a 3beta-hydroxysteroid-4alpha-carboxylate + NAD(P)+ = a 3-oxosteroid + CO2 + NAD(P)H [RN:R12366 R12406]
Reaction(KEGG)
Substrate
3beta-hydroxysteroid-4alpha-carboxylate [CPD:C22111];
NAD+ [CPD:C00003];
NADP+ [CPD:C00006]
Product
3-oxosteroid [CPD:C01876];
CO2 [CPD:C00011];
NADH [CPD:C00004];
NADPH [CPD:C00005]
Comment
The enzyme participates in the biosynthesis of several important sterols such as ergosterol and cholesterol. It is part of a three enzyme system that removes methyl groups from the C-4 position of steroid molecules. The first enzyme, EC 1.14.18.9, 4alpha-methylsterol monooxygenase, catalyses three successive oxidations of the methyl group, resulting in a carboxyl group; the second enzyme, EC 1.1.1.170, catalyses an oxidative decarboxylation that results in a reduction of the 3beta-hydroxy group at the C-3 carbon to an oxo group; and the last enzyme, EC 1.1.1.270, 3beta-hydroxysteroid 3-dehydrogenase, reduces the 3-oxo group back to a 3beta-hydroxyl. If a second methyl group remains at the C-4 position, this enzyme also catalyses its epimerization from 4beta to 4alpha orientation, so it could serve as a substrate for a second round of demethylation. cf. EC 1.1.1.418, plant 3beta-hydroxysteroid-4alpha-carboxylate 3-dehydrogenase (decarboxylating).
History
EC 1.1.1.170 created 1978, modified 2002, modified 2012, modified 2019
Pathway
ec00100  Steroid biosynthesis
ec01100  Metabolic pathways
ec01130  Biosynthesis of antibiotics
Orthology
K07748  sterol-4alpha-carboxylate 3-dehydrogenase (decarboxylating)
Genes
HSA: 50814(NSDHL)
PTR: 465960(NSDHL)
PPS: 100975917(NSDHL)
GGO: 101144867(NSDHL)
PON: 100172372(NSDHL)
NLE: 100596441(NSDHL)
MCC: 693333(NSDHL)
MCF: 102137627(NSDHL)
CSAB: 103232767(NSDHL)
RRO: 104654022(NSDHL)
RBB: 108513868(NSDHL)
CJC: 100398582(NSDHL)
SBQ: 101027738(NSDHL)
MMU: 18194(Nsdhl)
MCAL: 110287212(Nsdhl)
MPAH: 110313833(Nsdhl)
RNO: 309262(Nsdhl)
MUN: 110540271(Nsdhl)
CGE: 100764429(Nsdhl) 100768776
NGI: 103731179(Nsdhl)
HGL: 101716507(Nsdhl)
CCAN: 109695438(Nsdhl)
OCU: 100351296(NSDHL)
TUP: 102500582(NSDHL)
CFA: 481079(NSDHL)
VVP: 112912365(NSDHL)
AML: 100466780(NSDHL)
UMR: 103674163(NSDHL)
UAH: 113246811(NSDHL)
ORO: 101372464(NSDHL)
ELK: 111139213
FCA: 101096551(NSDHL)
PTG: 102959228(NSDHL)
PPAD: 109260189(NSDHL)
AJU: 106971243(NSDHL)
BTA: 616694(NSDHL)
BOM: 102276234(NSDHL)
BIU: 109555394(NSDHL)
BBUB: 102398147(NSDHL)
CHX: 102173170(NSDHL)
OAS: 101112792(NSDHL)
SSC: 100157894(NSDHL)
CFR: 102516550(NSDHL)
CDK: 105104051(NSDHL)
BACU: 103020703(NSDHL)
LVE: 103087463(NSDHL)
OOR: 101278134(NSDHL)
DLE: 111170601(NSDHL)
PCAD: 102989952(NSDHL)
ECB: 100063648(NSDHL)
EPZ: 103543383(NSDHL)
EAI: 106834275(NSDHL)
MYB: 102257708(NSDHL)
MYD: 102771848(NSDHL)
MNA: 107537970(NSDHL)
HAI: 109394344(NSDHL)
DRO: 112296338(NSDHL)
PALE: 102879960(NSDHL)
RAY: 107520609(NSDHL)
MJV: 108402575(NSDHL)
LAV: 100671133(NSDHL)
TMU: 101357526
MDO: 100026414(NSDHL)
SHR: 100914940(NSDHL)
PCW: 110199978(NSDHL)
OAA: 103171093(NSDHL)
GGA: 422302(NSDHL)
MGP: 100544971(NSDHL)
CJO: 107312870(NSDHL)
NMEL: 110402996(NSDHL)
APLA: 101790127(NSDHL)
ACYG: 106047509(NSDHL)
TGU: 100190543(NSDHL)
LSR: 110477979(NSDHL)
SCAN: 103816322(NSDHL)
GFR: 102041531(NSDHL)
FAB: 101814664(NSDHL)
PHI: 102100867(NSDHL)
PMAJ: 107204042(NSDHL)
CCAE: 111929260(NSDHL)
CCW: 104691595(NSDHL)
ETL: 114062107(NSDHL)
FPG: 101921503(NSDHL)
FCH: 102048326(NSDHL)
CLV: 102098029(NSDHL)
EGZ: 104127975(NSDHL)
NNI: 104021094(NSDHL)
ACUN: 113480109(NSDHL)
PADL: 103925887(NSDHL)
AAM: 106489838(NSDHL)
ASN: 102383140(NSDHL)
AMJ: 102562140(NSDHL)
PSS: 102447619(NSDHL)
CMY: 102945577(NSDHL)
CPIC: 101937255(NSDHL)
ACS: 100562829(nsdhl)
PVT: 110088403(NSDHL)
PBI: 103061373(NSDHL)
PMUR: 107293162(NSDHL)
TSR: 106555925(NSDHL)
PMUA: 114589018(NSDHL)
GJA: 107118212(NSDHL)
XLA: 108700163(nsdhl.S) 496236(nsdhl.L)
XTR: 550044(nsdhl)
NPR: 108795343(NSDHL)
DRE: 550369(nsdhl)
SRX: 107733445(nsdhl)
SANH: 107663101(nsdhl)
SGH: 107554660(nsdhl)
IPU: 108268902(nsdhl)
PHYP: 113542904(nsdhl)
AMEX: 103027604(nsdhl)
EEE: 113576547(nsdhl)
TRU: 101061359(nsdhl)
LCO: 104923201(nsdhl)
NCC: 104948435(nsdhl)
MZE: 101480912(nsdhl)
ONL: 100712373(nsdhl)
OLA: 101171010(nsdhl)
XMA: 102221009(nsdhl)
XCO: 114139190(nsdhl)
PRET: 103471168(nsdhl)
CVG: 107089032(nsdhl)
NFU: 107395696(nsdhl)
KMR: 108242032(nsdhl)
ALIM: 106515962(nsdhl)
AOCE: 111565582(nsdhl)
CSEM: 103391149(nsdhl)
POV: 109624038(nsdhl)
LCF: 108882885(nsdhl)
SDU: 111226496(nsdhl)
SLAL: 111652007(nsdhl)
HCQ: 109515829(nsdhl)
BPEC: 110163595(nsdhl)
MALB: 109968402(nsdhl)
ELS: 105025751(nsdhl)
SFM: 108925361(nsdhl)
PKI: 111843704(nsdhl)
LCM: 102355835(NSDHL)
CMK: 103177331(nsdhl)
RTP: 109917662(nsdhl)
SPU: 577670
APLC: 110983741
SKO: 100367277
PCAN: 112560719
LAK: 106150518
AQU: 100641976
CRE: CHLREDRAFT_58501(BHSD1)
MNG: MNEG_5826
APRO: F751_0534
SCE: YGL001C(ERG26)
ERC: Ecym_6178
KMX: KLMA_40026(ERG26)
NCS: NCAS_0A05210(NCAS0A05210)
NDI: NDAI_0K02600(NDAI0K02600)
TPF: TPHA_0K01550(TPHA0K01550)
TBL: TBLA_0E01630(TBLA0E01630)
TDL: TDEL_0F02190(TDEL0F02190)
KAF: KAFR_0F03320(KAFR0F03320)
PIC: PICST_71224(ERG26)
CAL: CAALFM_C406270CA(ERG26)
CDU: CD36_45790(ERG26)
SLB: AWJ20_2805(ERG26)
NCR: NCU02693
NTE: NEUTE1DRAFT119017(NEUTE1DRAFT_119017)
CMT: CCM_07037
MBE: MBM_00113
ANG: ANI_1_470134(An15g03090)
PBL: PAAG_11343(PAAG_01699)
PTE: PTT_14047
CNE: CNJ00460
CNB: CNBJ3060
ABP: AGABI1DRAFT113991(AGABI1DRAFT_113991)
ABV: AGABI2DRAFT193828(AGABI2DRAFT_193828)
MGL: MGL_3391
MRT: MRET_1855
SPAR: SPRG_01499
 » show all
Taxonomy
Reference
1  [PMID:5791927]
  Authors
Sharpless KB, Snyder TE, Spencer TA, Maheshwari KK, Nelson JA
  Title
Biological demethylation of 4,4-dimethyl sterols, Evidence for enzymic epimerization of the 4beta-methyl group prior to its oxidative removal.
  Journal
J Am Chem Soc 91:3394-6 (1969)
Reference
2  [PMID:4401584]
  Authors
Rahimtula AD, Gaylor JL.
  Title
Partial purification of a microsomal sterol 4 -carboxylic acid decarboxylase.
  Journal
J Biol Chem 247:9-15 (1972)
Reference
3  [PMID:7430141]
  Authors
Brady DR, Crowder RD, Hayes WJ.
  Title
Mixed function oxidases in sterol metabolism. Source of reducing equivalents.
  Journal
J Biol Chem 255:10624-9 (1980)
Reference
4  [PMID:9811880]
  Authors
Gachotte D, Barbuch R, Gaylor J, Nickel E, Bard M
  Title
Characterization of the Saccharomyces cerevisiae ERG26 gene encoding the C-3 sterol dehydrogenase (C-4 decarboxylase) involved in sterol biosynthesis.
  Journal
Proc Natl Acad Sci U S A 95:13794-9 (1998)
DOI:10.1073/pnas.95.23.13794
  Sequence
[sce:YGL001C]
Reference
5  [PMID:14506130]
  Authors
Caldas H, Herman GE
  Title
NSDHL, an enzyme involved in cholesterol biosynthesis, traffics through the Golgi and accumulates on ER membranes and on the surface of lipid droplets.
  Journal
Hum Mol Genet 12:2981-91 (2003)
DOI:10.1093/hmg/ddg321
  Sequence
[hsa:50814]
Other DBs
ExplorEnz - The Enzyme Database: 1.1.1.170
IUBMB Enzyme Nomenclature: 1.1.1.170
ExPASy - ENZYME nomenclature database: 1.1.1.170
BRENDA, the Enzyme Database: 1.1.1.170
CAS: 71822-23-6

KEGG   REACTION: R12404Help
Entry
R12404                      Reaction                               

Name
4alpha-carboxy-5alpha-cholesta-8,24-dien-3beta-ol:NADP+ 3-oxidoreductase (decarboxylating)
Definition
4alpha-Carboxy-5alpha-cholesta-8,24-dien-3beta-ol + NADP+ <=> Zymosterone + NADPH + H+ + CO2
Equation
Reaction class
RC03536  C22112_C22136
Enzyme
Pathway
rn00100  Steroid biosynthesis
rn01100  Metabolic pathways
rn01130  Biosynthesis of antibiotics
Module
M00101  Cholesterol biosynthesis, squalene 2,3-epoxide => cholesterol
Orthology
K07748  sterol-4alpha-carboxylate 3-dehydrogenase (decarboxylating) [EC:1.1.1.170]

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