KEGG ORTHOLOGY: K16813

ORTHOLOGY: K16813

Entry

K16813                      KO

Symbol

PNPLA1

Name

omega-hydroxyceramide transacylase [EC:2.3.1.296]

Disease

H00734

Autosomal recessive congenital ichthyosis

Brite

KEGG Orthology (KO) [BR:ko00001]
09190 Not Included in Pathway or Brite
  09191 Unclassified: metabolism
   99980 Enzymes with EC numbers
    K16813  PNPLA1; omega-hydroxyceramide transacylase
Enzymes [BR:ko01000]
2. Transferases
  2.3  Acyltransferases
   2.3.1  Transferring groups other than aminoacyl groups
    2.3.1.296  omega-hydroxyceramide transacylase
     K16813  PNPLA1; omega-hydroxyceramide transacylase

Genes

HSA:	285848(PNPLA1)

PTR:	471991(PNPLA1)

PPS:	100981874(PNPLA1)

GGO:	101149146(PNPLA1)

PON:	100450832(PNPLA1)

NLE:	100596271(PNPLA1)

HMH:	116459954(PNPLA1)

MCC:	100426523(PNPLA1)

MCF:	102142984(PNPLA1)

MTHB:

126952765

MNI:	105474519(PNPLA1)

CSAB:

103221593(PNPLA1)

CATY:

105593818(PNPLA1)

PANU:

101025463(PNPLA1)

TGE:	112623430(PNPLA1)

MLEU:

105544224(PNPLA1)

RRO:	104682872(PNPLA1)

RBB:	108528307(PNPLA1)

TFN:	117087124(PNPLA1)

PTEH:

111542888(PNPLA1)

CANG:

105514172(PNPLA1)

CJC:	100415082(PNPLA1)

SBQ:	101044461(PNPLA1)

CIMI:

108300000(PNPLA1)

CSYR:

103265296(PNPLA1)

MMUR:

105861455(PNPLA1)

LCAT:

123632080(PNPLA1)

PCOQ:

105812192(PNPLA1)

OGA:	100957737(PNPLA1)

MMU:	433091(Pnpla1)

MCAL:

110283928(Pnpla1)

MPAH:

110338533(Pnpla1)

RNO:	361812(Pnpla1)

MCOC:

116075243(Pnpla1)

ANU:	117724871(Pnpla1)

MUN:	110546583(Pnpla1)

CGE:	100763234(Pnpla1)

MAUA:

101831359(Pnpla1)

PROB:

127223908(Pnpla1)

PLEU:

114706161(Pnpla1)

MORG:

121435720(Pnpla1)

MFOT:

126502532

AAMP:

119823834(Pnpla1)

NGI:	103739726(Pnpla1)

HGL:	101713574(Pnpla1)

CPOC:

100728999(Pnpla1)

CCAN:

109689861(Pnpla1)

DORD:

105995744(Pnpla1)

DSP:	122111147(Pnpla1)

PLOP:

125353506(Pnpla1)

NCAR:

124987961

MMMA:

107143844(Pnpla1)

OCU:

100339908

OPI:	101521491(PNPLA1)

TUP:	102494197(PNPLA1)

GVR:	103607576(PNPLA1)

CFA:	481763(PNPLA1)

CLUD:

112641486(PNPLA1)

VVP:	112913935(PNPLA1)

VLG:	121495666(PNPLA1)

NPO:	129512665(PNPLA1)

AML:	100464720(PNPLA1)

UMR:	103665449(PNPLA1)

UAH:	113243858(PNPLA1)

UAR:	123779568(PNPLA1)

ELK:

111152378

LLV:

125103227

MPUF:

101685859(PNPLA1)

MNP:	132017211(PNPLA1)

MLK:	131834186(PNPLA1)

NVS:	122909994(PNPLA1)

ORO:	101376548(PNPLA1)

EJU:	114222073(PNPLA1)

ZCA:	113927076(PNPLA1)

MLX:	118001200(PNPLA1)

NSU:	110575676(PNPLA1)

LWW:	102727615(PNPLA1)

FCA:	101091788(PNPLA1)

PYU:	121029358(PNPLA1)

PBG:	122490352(PNPLA1)

PVIV:

125165871(PNPLA1)

LRUF:

124529284

PTG:	102966751(PNPLA1)

PPAD:

109271519(PNPLA1)

PUC:

125938316

AJU:

106972772

HHV:	120233831(PNPLA1)

BTA:	786888(PNPLA1)

BOM:	102270258(PNPLA1)

BIU:	109577451(PNPLA1)

BBUB:

102398763(PNPLA1)

BBIS:

104994942(PNPLA1)

CHX:	102189446(PNPLA1)

OAS:	101120362(PNPLA1)

BTAX:

128055138(PNPLA1)

ODA:	120870207(PNPLA1)

CCAD:

122429581(PNPLA1)

MBEZ:

129543798(PNPLA1)

SSC:	100154642(PNPLA1)

CFR:	102512995(PNPLA1)

CBAI:

105064172(PNPLA1)

CDK:	105101336(PNPLA1)

VPC:	102528081(PNPLA1)

BACU:

103007961(PNPLA1)

BMUS:

118904197(PNPLA1)

LVE:	103087327(PNPLA1)

OOR:	101285071(PNPLA1)

DLE:	111178608(PNPLA1)

PCAD:

102990565(PNPLA1)

ECB:	100063661(PNPLA1)

EPZ:	103563196(PNPLA1)

EAI:	106824602(PNPLA1)

MYB:	102247326(PNPLA1)

MYD:	102756555(PNPLA1)

MMYO:

118660076(PNPLA1)

MLF:	102442402(PNPLA1)

PKL:	118705841(PNPLA1)

EFUS:

103295576(PNPLA1)

MNA:	107545587(PNPLA1)

DRO:	112302420(PNPLA1)

SHON:

118998969(PNPLA1)

AJM:	119050074(PNPLA1)

PDIC:

114495514(PNPLA1)

PHAS:

123822263(PNPLA1)

MMF:	118621825(PNPLA1)

PPAM:

129067009(PNPLA1)

HAI:	109395975(PNPLA1)

RFQ:	117015543(PNPLA1)

PALE:

102898567(PNPLA1)

PGIG:

120597573(PNPLA1)

PVP:	105288542(PNPLA1)

RAY:	107514707(PNPLA1)

MJV:	108390133(PNPLA1)

TOD:	119253297(PNPLA1)

SARA:

101540597(PNPLA1)

SETR:

125995823(PNPLA1)

LAV:	100672854(PNPLA1)

TMU:

101340560

ETF:	101658379(PNPLA1)

DNM:	101433422(PNPLA1)

MDO:	100028911(PNPLA1)

GAS:	123247070(PNPLA1)

SHR:	100927942(PNPLA1)

AFZ:

127561335

PCW:	110217314(PNPLA1)

OAA:	114813289(PNPLA1)

GGA:	428259(PNPLA1)

PCOC:

116236969 116236970(PNPLA1)

MGP:	100542403 100542559(PNPLA1)

CJO:	107324687(PNPLA1) 107324688

TPAI:

128087973(PNPLA1) 128088193

LMUT:

125684261(PNPLA1)

NMEL:

110388084 110388095(PNPLA1)

APLA:

119713935(PNPLA1)

ACYG:

106048433(PNPLA1)

CATA:

118260418(PNPLA1)

AFUL:

116498701(PNPLA1)

TGU:	100225038(PNPLA1) 115498565

LSR:	110473035(PNPLA1)

SCAN:

103822414(PNPLA1) 108963115

PMOA:

120497628(PNPLA1)

OTC:	121345513(PNPLA1) 121345514

PRUF:

121349761(PNPLA1)

GFR:	102032738 102041930(PNPLA1)

FAB:	101809281(PNPLA1) 101817044

OMA:	130263836(PNPLA1)

PHI:	102109501 102113209(PNPLA1)

PMAJ:

107214760 107215020(PNPLA1)

CCAE:

111939875(PNPLA1) 111939878

CCW:	104692425(PNPLA1) 109145293

CBRC:

103619070(PNPLA1)

ETL:	114064790(PNPLA1)

ZAB:	102065438(PNPLA1)

ACHL:

103807326

SVG:	106850933(PNPLA1)

MMEA:

130578840(PNPLA1)

HRT:	120762871(PNPLA1) 120762965

FPG:	101920072(PNPLA1)

FCH:	102058013 114018075

CLV:	102097663(PNPLA1) 110364654

EGZ:	104131423(PNPLA1)

NNI:	104018436 104018437

PCRI:

104029985

PLET:

104621756(PNPLA1)

EHS:

104509992

PCAO:

104042537

ACUN:

113489012(PNPLA1)

TALA:

104359130(PNPLA1)

PADL:

103925952 103925953(PNPLA1)

AFOR:

103908270(PNPLA1)

ACHC:

115335218(PNPLA1)

HALD:

104315359(PNPLA1)

HLE:	104842451(PNPLA1)

AGEN:

126052237

GCL:

127026112

CCRI:

104167251(PNPLA1)

CSTI:

104557167(PNPLA1)

MUI:	104538299(PNPLA1)

BREG:

FGA:

GSTE:

LDI:

MNB:	103781500(PNPLA1)

OHA:	104328255(PNPLA1)

NNT:

104405411

SHAB:

115602465 115602466

DPUB:

104304948(PNPLA1)

PGUU:

104468912

CPEA:

104392036(PNPLA1)

AVIT:

104277765(PNPLA1)

CVF:	104292376(PNPLA1)

RTD:	128900454(PNPLA1)

TEO:

104383376

BRHI:

104496009

AROW:

112961441(PNPLA1)

NPD:	112944597(PNPLA1)

TGT:	104566832(PNPLA1)

DNE:	112990369(PNPLA1)

SCAM:

104142550(PNPLA1)

ASN:	102381144 102381898 102387352 102387599 112550535

AMJ:	102574209 102574443

CPOO:

109317520 109317522

GGN:	109295081 109295082

PSS:	102452905 102453155

CMY:	102931927 102932153

CCAY:

125624991 125624993

DCC:	119846262 119846540

CPIC:

101931701 101931995

TST:	117876544 117876814

CABI:

116839152 116839161

MRV:	120404008 120404009

ACS:	100553180 100568175

ASAO:

PVT:

SUND:

VKO:

PMUA:

PRAF:

ZVI:

HCG:

GJA:	107110977(PNPLA1)

XLA:	108708055(pnpla1.L) 108709386(pnpla1.S)

XTR:	100497390(pnpla1)

NPR:

RTEM:

BBUF:

BGAR:

HOC:

LERI:

PMRN:

PJA:

Reference

PMID:16799181

Authors

Wilson PA, Gardner SD, Lambie NM, Commans SA, Crowther DJ

Title

Characterization of the human patatin-like phospholipase family.

Journal

J Lipid Res 47:1940-9 (2006)
DOI:10.1194/jlr.M600185-JLR200

Reference

PMID:28248318

Authors

Ohno Y, Kamiyama N, Nakamichi S, Kihara A

Title

PNPLA1 is a transacylase essential for the generation of the skin barrier lipid omega-O-acylceramide.

Journal

Nat Commun 8:14610 (2017)
DOI:10.1038/ncomms14610

Sequence

[hsa:285848]

LinkDB

DBGET integrated database retrieval system