EC                Enzyme                                 

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)
Acting on the CH-OH group of donors;
With NAD+ or NADP+ as acceptor
3beta-hydroxysteroid-4alpha-carboxylate:NAD(P)+ 3-oxidoreductase (decarboxylating)
a 3beta-hydroxysteroid-4alpha-carboxylate + NAD(P)+ = a 3-oxosteroid + CO2 + NAD(P)H [RN:R12366 R12406]
R12366 > R04483;
R12406 > R07149 R07494 R12404;
(other) R04328
3beta-hydroxysteroid-4alpha-carboxylate [CPD:C22111];
NAD+ [CPD:C00003];
NADP+ [CPD:C00006]
3-oxosteroid [CPD:C01876];
CO2 [CPD:C00011];
NADH [CPD:C00004];
NADPH [CPD:C00005]
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, 4alpha-methylsterol monooxygenase, catalyses three successive oxidations of the methyl group, resulting in a carboxyl group; the second enzyme, EC, 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, 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, plant 3beta-hydroxysteroid-4alpha-carboxylate 3-dehydrogenase (decarboxylating).
EC created 1978, modified 2002, modified 2012, modified 2019
ec00100  Steroid biosynthesis
ec01100  Metabolic pathways
ec01110  Biosynthesis of secondary metabolites
K07748  sterol-4alpha-carboxylate 3-dehydrogenase (decarboxylating)
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)
CATY: 105577846(NSDHL)
PANU: 101003162(NSDHL)
RRO: 104654022(NSDHL)
RBB: 108513868(NSDHL)
TFN: 117078175(NSDHL)
PTEH: 111535852(NSDHL)
CJC: 100398582(NSDHL)
SBQ: 101027738(NSDHL)
MMUR: 105884688(NSDHL)
MMU: 18194(Nsdhl)
MCAL: 110287212(Nsdhl)
MPAH: 110313833(Nsdhl)
RNO: 309262(Nsdhl)
MCOC: 116085853(Nsdhl)
MUN: 110540271(Nsdhl)
CGE: 100764429(Nsdhl)
PLEU: 114687208(Nsdhl)
NGI: 103731179(Nsdhl)
HGL: 101716507(Nsdhl)
CCAN: 109695438(Nsdhl)
OCU: 100351296(NSDHL)
OPI: 101523731(NSDHL)
TUP: 102500582(NSDHL)
CFA: 481079(NSDHL)
VVP: 112912365(NSDHL)
VLG: 121483462(NSDHL)
AML: 100466780(NSDHL)
UMR: 103674163(NSDHL)
UAH: 113246811(NSDHL)
ORO: 101372464(NSDHL)
ELK: 111139213
MPUF: 101678660(NSDHL)
EJU: 114200013(NSDHL)
MLX: 118015061(NSDHL)
FCA: 101096551(NSDHL)
PYU: 121025059(NSDHL)
PBG: 122477763(NSDHL)
PTG: 102959228(NSDHL)
PPAD: 109260189(NSDHL)
AJU: 106971243(NSDHL)
HHV: 120222745(NSDHL)
BTA: 616694(NSDHL)
BOM: 102276234(NSDHL)
BIU: 109555394(NSDHL)
BBUB: 102398147(NSDHL)
CHX: 102173170(NSDHL)
OAS: 101112792(NSDHL)
ODA: 120881834(NSDHL)
CCAD: 122435188(NSDHL)
SSC: 100157894(NSDHL)
CFR: 102516550(NSDHL)
CBAI: 105070047(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)
MMYO: 118654030(NSDHL)
MNA: 107537970(NSDHL)
HAI: 109394344(NSDHL)
DRO: 112296338(NSDHL)
SHON: 118996037(NSDHL)
AJM: 119040353(NSDHL)
MMF: 118636496(NSDHL)
PALE: 102879960(NSDHL)
PGIG: 120614230(NSDHL)
RAY: 107520609(NSDHL)
MJV: 108402575(NSDHL)
TOD: 119245819(NSDHL)
LAV: 100671133(NSDHL)
TMU: 101357526
MDO: 100026414(NSDHL)
SHR: 100914940(NSDHL)
PCW: 110199978(NSDHL)
OAA: 103171093(NSDHL)
GGA: 422302(NSDHL)
PCOC: 116229717(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)
PMOA: 120512703(NSDHL)
OTC: 121346437(NSDHL)
PRUF: 121362827(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)
AROW: 112975809(NSDHL)
NPD: 112946970(NSDHL)
DNE: 112983256(NSDHL)
ASN: 102383140(NSDHL)
AMJ: 102562140(NSDHL)
CPOO: 109313657(NSDHL)
GGN: 109300684(NSDHL)
PSS: 102446000(ZNF185) 102447619(NSDHL)
CMY: 102942264(ZNF185) 102945577(NSDHL)
CPIC: 101937255(ZNF185) 112058643(NSDHL)
TST: 117882424(NSDHL)
CABI: 116817089(NSDHL)
ACS: 100562829(nsdhl)
PVT: 110088403(NSDHL)
PBI: 103061373(NSDHL)
PMUR: 107293162(NSDHL)
TSR: 106555925(NSDHL)
PGUT: 117669520(NSDHL)
PMUA: 114589018(NSDHL)
ZVI: 118084372(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)
CGOB: 115014428(nsdhl)
ELY: 117260605(nsdhl)
PLEP: 121948179(nsdhl)
SLUC: 116064271(nsdhl)
ECRA: 117951569(nsdhl)
PFLV: 114562747(nsdhl)
GAT: 120818015(nsdhl)
PPUG: 119211431(nsdhl)
MSAM: 119888821(nsdhl) 119919306
CUD: 121515971(nsdhl)
MZE: 101480912(nsdhl)
ONL: 100712373(nsdhl)
OAU: 116324542(nsdhl)
OLA: 101171010(nsdhl)
OML: 112153776(nsdhl)
XMA: 102221009(nsdhl)
XCO: 114139190(nsdhl)
XHE: 116714396(nsdhl)
PRET: 103471168(nsdhl)
CVG: 107089032(nsdhl)
CTUL: 119792450(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)
XGL: 120785536(nsdhl)
HCQ: 109515829(nsdhl)
BPEC: 110163595(nsdhl)
MALB: 109968402(nsdhl)
OMY: 110489156 110504568(nsdhl)
SNH: 120047904 120062403(nsdhl)
ELS: 105025751(nsdhl)
SFM: 108925361(nsdhl)
PKI: 111843704(nsdhl)
AANG: 118236510(nsdhl)
LOC: 102686211(nsdhl)
PSPA: 121295893 121318722(nsdhl)
LCM: 102355835(NSDHL)
CMK: 103177331(nsdhl)
RTP: 109917662(nsdhl)
BFO: 118419129
BBEL: 109470548
SPU: 577670
APLC: 110983741
SKO: 100367277
PCAN: 112560719
BGT: 106068123
GAE: 121376237
OSN: 118761140
LAK: 106150518
AQU: 100641976
APRO: F751_0534
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)
CDU: CD36_45790(ERG26)
SLB: AWJ20_2805(ERG26)
BNN: FOA43_000837(ERG26)
BBRX: BRETT_001264(ERG26)
NCR: NCU02693
CMT: CCM_07037
MBE: MBM_00113
ANG: ANI_1_470134(An15g03090)
PBL: PAAG_11343(PAAG_01699)
PTE: PTT_14047
CNE: CNJ00460
TASA: A1Q1_01819
MGL: MGL_3391
MRT: MRET_1855
DFA: DFA_04893
SPAR: SPRG_01499
 » show all
1  [PMID:5791927]
Sharpless KB, Snyder TE, Spencer TA, Maheshwari KK, Nelson JA
Biological demethylation of 4,4-dimethyl sterols, Evidence for enzymic epimerization of the 4beta-methyl group prior to its oxidative removal.
J Am Chem Soc 91:3394-6 (1969)
2  [PMID:4401584]
Rahimtula AD, Gaylor JL.
Partial purification of a microsomal sterol 4 -carboxylic acid decarboxylase.
J Biol Chem 247:9-15 (1972)
3  [PMID:7430141]
Brady DR, Crowder RD, Hayes WJ
Mixed function oxidases in sterol metabolism. Source of reducing equivalents.
J Biol Chem 255:10624-9 (1980)
4  [PMID:9811880]
Gachotte D, Barbuch R, Gaylor J, Nickel E, Bard M
Characterization of the Saccharomyces cerevisiae ERG26 gene encoding the C-3 sterol dehydrogenase (C-4 decarboxylase) involved in sterol biosynthesis.
Proc Natl Acad Sci U S A 95:13794-9 (1998)
5  [PMID:14506130]
Caldas H, Herman GE
NSDHL, an enzyme involved in cholesterol biosynthesis, traffics through the Golgi and accumulates on ER membranes and on the surface of lipid droplets.
Hum Mol Genet 12:2981-91 (2003)
Other DBs
ExplorEnz - The Enzyme Database:
IUBMB Enzyme Nomenclature:
ExPASy - ENZYME nomenclature database:
BRENDA, the Enzyme Database:
CAS: 71822-23-6

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