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Giving unique and meaningful names to every human gene

Following a 9 months email discussion the following decisions have been made about the nomenclature of this family:

HGNC aims to provide a unique gene symbol, which will be used in all publications. Looking at the publication history of this family HGNC is reluctant to approve the NLR stem for the most highly published genes, as inevitably these genes will continue to be referred to as CARD4/NOD1 etc. Therefore HGNC recommends that the following designations should be used by the community in future publications:

Human gene details in BLUE (approved symbols in upper case), Mouse gene details in BLACK (approved symbols in upper and lower case).

Visit http://www.genenames.org/genefamily/nlr.php for active links to the human gene details

New approved symbol New approved name

Previous approved symbol

Additional alias

Other names and aliases

Domain organization

Entrez Gene ID

Protein sequence

CIITA class II, major histocompatibility complex, transactivator

MHC2TA

NLRA

C2TA

(CARD)-AD-NACHT-NAD-LRR

4261

NP_000237

CIIta class II, major histocompatibility complex, transactivator  

Nlra

C2TA

(CARD)-AD-NACHT-NAD-LRR

12265

NP_031601

NAIP NLR family, apoptosis inhibitory protein

BIRC1

NLRB1

CLR5.1

BIR3x-NACHT-NAD?-LRR

4671

NP_004527

Naip1 NLR family, apoptosis inhibitory protein 1

Birc1a

Nlrb1a

BIR3x-NACHT-NAD?-LRR

17940

NP_03269

Naip2 NLR family, apoptosis inhibitory protein 2

Birc1b

Nlrb1b

Naip-rs6

BIR3x-NACHT-NAD?-LRR

17948

NP_035002

Naip3 NLR family, apoptosis inhibitory protein 3

Birc1c

Nlrb1c

Naip-rs5

BIR3x-NACHT-NAD?-LRR

17949

U66328

Naip4 NLR family, apoptosis inhibitory protein 4

Birc1d

Nlrb1d

Naip-rs2

BIR3x-NACHT-NAD?-LRR

17950

U66325

Naip5 NLR family, apoptosis inhibitory protein 5

Birc1e

Nlrb1e

Naip-rs3

BIR3x-NACHT-NAD?-LRR

17951

NP_03500

Naip6 NLR family, apoptosis inhibitory protein 6

Birc1f

Nlrb1f

Naip-rs4

BIR3x-NACHT-NAD?-LRR

17952

NP_035001

Naip7 NLR family, apoptosis inhibitory protein 7

Birc1g

Nlrb1g

BIR3x-NACHT-NAD?-LRR

53880

AAF82749

NOD1 nucleotide-binding oligomerization domain containing 1

CARD4

NLRC1

CLR7.1

CARD-NACHT-NAD-LRR

10392

NP_006083

Nod1 nucleotide-binding oligomerization domain containing 1

Card4

Nlrc1

CARD-NACHT-NAD-LRR

107607

NP_766317

NOD2 nucleotide-binding oligomerization domain containing 2

CARD15

NLRC2

CD, BLAU, IBD1, PSORAS1; CLR16.3

CARD2x-NACHT-NAD-LRR

64127

NP_071445

Nod2 nucleotide-binding oligomerization domain containing 2

Card15

Nlrc2

CARD2x-NACHT-NAD-LRR

257632

NP_665856

NLRC3 NLR family, CARD domain containing 3

None

 

NOD3; CLR16.2

CARD-NACHT-NAD-LRR

197358

NP_849172

Nlrc3

NLR family, CARD domain containing 3

None

 

CLR16.2

CARD-NACHT-NAD-LRR

268857

XP_484522

NLRC4 NLR family, CARD domain containing 4

CARD12

 

CLAN; CLR2.1, IPAF

CARD-NACHT-NAD-LRR

58484

NP_067032

Nlrc4

NLR family, CARD domain containing 4

Card12

 

CLAN, Ipaf

CARD-NACHT-NAD-LRR

268973

XP_197335

NLRC5

NLR family, CARD domain containing 5

None

 

NOD27; CLR16.1

CARD-NACHT-NAD-LRR

84166

NP_115582

Nlrc5

NLR family, CARD domain containing 5

None

 

none

CARD-NACHT-NAD-LRR

434341

XP_486147

NLRX1

NLR family member X1

None

 

NOD9; CLR11.3

X-NACHT-NAD-LRR

79671

NP_078894

Nlrx1 NLR family member X1

None

 

none

X-NACHT-NAD-LRR

270151

NP_848507

NLRP1

NLR family, pyrin domain containing 1

NALP1

 

DEFCAP; NAC; CARD7; CLR17.1

PYD-NACHT-NAD-LRR-FIIND-CARD

22861

NP_127497

Nlrp1a

NLR family, pyrin domain containing 1A

Nalp1

 

Nalp1a

NACHT-NAD-LRR-FIIND-CARD

195046

NP_001004142

Nlrp1b

NLR family, pyrin domain containing 1B

None

 

Nalp1b

NACHT-?-LRR

637515

NP_001034769

Nlrp1c

NLR family, pyrin domain containing 1C

None

 

Nalp1c

627984

NP_001034323

NLRP2

NLR family, pyrin domain containing 2

NALP2

 

PYPAF2; NBS1; PAN1; CLR19.9

PYD-NACHT-NAD-LRR

55655

NP_060322

Nlrp2

NLR family, pyrin domain containing 2

Nalp2

 

Pypaf2, Nbs1, Pan1

PYD-NACHT-NAD-LRR

232827

NP_808358

NLRP3

NLR family, pyrin domain containing 3

CIAS1

 

PYPAF1, Cryopyrin; CLR1.1, NALP3

PYD-NACHT-NAD-LRR

114548

NP_00488

Nlrp3

NLR family, pyrin domain containing 3

Cias1

 

Pypaf1, Mmig1, Nalp3

PYD-NACHT-NAD-LRR

216799

NP_665826

NLRP4

NLR family, pyrin domain containing 4

NALP4

 

PYPAF4;PAN2; RNH2; CLR19.5

PYD-NACHT-NAD-LRR

147945

NP_604393

Nlrp4a

NLR family, pyrin domain containing 4A

Nalp4a

 

Nalp-eta, Nalp9D

PYD-NACHT-NAD-LRR

243880

NP_766484

Nlrp4b

NLR family, pyrin domain containing 4B

Nalp4b

 

Nalp-gamma, Nalp9E

PYD-NACHT-NAD-LRR

210045

NP_766069

Nlrp4c

NLR family, pyrin domain containing 4C

Nalp4c

 

Nalp-alpha, Rnh2

PYD-NACHT-NAD-LRR

83564

NP_113566

Nlrp4d

NLR family, pyrin domain containing 4D

Nalp4d

 

Nalp-beta

PYD-NACHT-NAD-LRR

384752

AAU06316

Nlrp4e

NLR family, pyrin domain containing 4E

Nalp4e

 

Nalp-epsilon

PYD-NACHT-NAD-LRR

446099

NP_001004194

Nlrp4f

NLR family, pyrin domain containing 4F

Nalp4f

 

Nalp-kappa, Nalp9F

PYD-NACHT-NAD-LRR

97895

NP_780499

Nlrp4g

NLR family, pyrin domain containing 4G

Nalp4g

 

none

PYD-NACHT-NAD-LRR

235779

AAH80833

NLRP5

NLR family, pyrin domain containing 5

NALP5

 

PYPAF8; MATER, PAN11; CLR19.8

PYD-NACHT-NAD-LRR

126206

NP_703148

Nlrp5

NLR family, pyrin domain containing 5

Nalp5

 

Mater, Op1

NACHT-NAD-LRR

23968

NP_035990

NLRP6

NLR family, pyrin domain containing 6

NALP6

 

PYPAF5; PAN3; CLR11.4

PYD-NACHT-NAD-LRR

171389

NP_612202

Nlrp6

NLR family, pyrin domain containing 6

Nalp6

 

none

PYD-NACHT-NAD-LRR

101613

AY355343

NLRP7

NLR family, pyrin domain containing 7

NALP7

 

PYPAF3; NOD12; PAN7; CLR19.4

PYD-NACHT-NAD-LRR

199713

NP_996611

NLRP8

NLR family, pyrin domain containing 8

NALP8

 

PAN4; NOD16; CLR19.2

PYD-NACHT-NAD-LRR

126205

NP_789781

NLRP9

NLR family, pyrin domain containing 9

NALP9

 

NOD6; ; PAN12; CLR19.1

PYD-NACHT-NAD-LRR

338321

NP_789790

Nlrp9a

NLR family, pyrin domain containing 9A

Nalp9a

 

Nalp-theta

PYD-NACHT-NAD-LRR

23300

NP_918945

Nlrp9b

NLR family, pyrin domain containing 9B

Nalp9b

 

Nalp-delta

PYD-NACHT-NAD-LRR

243874

NP_918947

Nlrp9c

NLR family, pyrin domain containing 9C

Nalp9c

 

Nalp-zeta

PYD-NACHT-NAD-LRR

330490

NP_918951

NLRP10

NLR family, pyrin domain containing 10

NALP10

 

PAN5; NOD8; Pynod; CLR11.1

PYD-NACHT-NAD

338322

NP_789791

Nlrp10

NLR family, pyrin domain containing 10

Nalp10

 

Pynod

PYD-NACHT-NAD

244202

NP_780741

NLRP11

NLR family, pyrin domain containing 11

NALP11

 

PYPAF6; NOD17; PAN10; CLR19.6

PYD-NACHT-NAD-LRR

204801

NP_659444

NLRP12

NLR family, pyrin domain containing 12

NALP12

 

PYPAF7; Monarch1; RNO2; PAN6; CLR19.3

PYD-NACHT-NAD-LRR

91662

NP_653288

Nlrp12

NLR family, pyrin domain containing 12

Nalp12

 

none

PYD-NACHT-NAD-LRR

378425

XP_355971

NLRP13

NLR family, pyrin domain containing 13

NALP13

 

NOD14; PAN13; CLR19.7

PYD-NACHT-NAD-LRR

126204

NP_78978

NLRP14

NLR family, pyrin domain containing 14

NALP14

 

NOD5; PAN8; ; CLR11.2

PYD-NACHT-NAD-LRR

338323

NP_789792

Nlrp14

NLR family, pyrin domain containing 14

Nalp14

 

Nalp-iota, GC-LRR,

PYD-NACHT-NAD-LRR

76858 

NP_001002894

Abbreviations: CARD, caspase activating and recruitment domain; LRR, leucine-rich repeat; NACHT, domain present in NAIP, CIITA, HET-E, TP-1; BIR, baculovirus inhibitor of apoptosis repeat; PYD, pyrin domain.

Links to previous pages

NACHT domain and LRR First Questionnaire discussion page

NACHT domain and LRR Second Questionnaire discussion page

NACHT domain and LRR Third Questionnaire discussion page

NACHT domain and LRR final discussion page


The work of the HGNC is supported by National Human Genome Research Institute (NHGRI) grant P41 HG03345 and Wellcome Trust grant 081979/Z/07/Z.