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Protein

Baculoviral IAP repeat-containing protein 1

Gene

NAIP

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Anti-apoptotic protein which acts by inhibiting the activities of CASP3, CASP7 and CASP9. Can inhibit the autocleavage of pro-CASP9 and cleavage of pro-CASP3 by CASP9. Capable of inhibiting CASP9 autoproteolysis at 'Asp-315' and decreasing the rate of auto proteolysis at 'Asp-330'. Acts as a mediator of neuronal survival in pathological conditions. Prevents motor-neuron apoptosis induced by a variety of signals. Possible role in the prevention of spinal muscular atrophy that seems to be caused by inappropriate persistence of motor-neuron apoptosis: mutated or deleted forms of NAIP have been found in individuals with severe spinal muscular atrophy.
Acts as a sensor component of the NLRC4 inflammasome that specifically recognizes and binds needle protein CprI from pathogenic bacteria C.violaceum. Association of pathogenic bacteria proteins drives in turn drive assembly and activation of the NLRC4 inflammasome, promoting caspase-1 activation, cytokine production and macrophage pyroptosis. The NLRC4 inflammasome is activated as part of the innate immune response to a range of intracellular bacteria such as C.violaceum and L.pneumophila.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi315Zinc1
Metal bindingi318Zinc1
Metal bindingi335Zinc1
Metal bindingi342Zinc1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi473 – 478ATPBy similarity6

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionProtease inhibitor, Thiol protease inhibitor
Biological processApoptosis, Immunity, Inflammatory response, Innate immunity
LigandATP-binding, Metal-binding, Nucleotide-binding, Zinc

Enzyme and pathway databases

SignaLinkiQ13075
SIGNORiQ13075

Protein family/group databases

MEROPSiI32.001

Names & Taxonomyi

Protein namesi
Recommended name:
Baculoviral IAP repeat-containing protein 1
Alternative name(s):
Neuronal apoptosis inhibitory protein
Gene namesi
Name:NAIP
Synonyms:BIRC1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 5

Organism-specific databases

EuPathDBiHostDB:ENSG00000249437.7
HGNCiHGNC:7634 NAIP
MIMi600355 gene
neXtProtiNX_Q13075

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi476K → T: Prevents the proper cleavage of pro-CASP9, but does not inhibit the cleavage of pro-CASP3 by CASP9. 1 Publication1

Organism-specific databases

DisGeNETi4671
MalaCardsiNAIP
OpenTargetsiENSG00000249437
Orphaneti83330 Proximal spinal muscular atrophy type 1
83418 Proximal spinal muscular atrophy type 2
83419 Proximal spinal muscular atrophy type 3
PharmGKBiPA162396805

Polymorphism and mutation databases

BioMutaiNAIP
DMDMi109940027

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00001223411 – 1403Baculoviral IAP repeat-containing protein 1Add BLAST1403

Proteomic databases

PaxDbiQ13075
PeptideAtlasiQ13075
PRIDEiQ13075

PTM databases

iPTMnetiQ13075
PhosphoSitePlusiQ13075

Expressioni

Tissue specificityi

Expressed in motor neurons, but not in sensory neurons. Found in liver and placenta, and to a lesser extent in spinal cord.

Gene expression databases

BgeeiENSG00000249437
CleanExiHS_NAIP
ExpressionAtlasiQ13075 baseline and differential
GenevisibleiQ13075 HS

Organism-specific databases

HPAiHPA042438

Interactioni

Subunit structurei

Interacts (via NACHT domain) with APAF1 (via CARD and NACHT domains). Interacts with C.violaceum needle protein CprI.3 Publications

Protein-protein interaction databases

BioGridi110752, 9 interactors
CORUMiQ13075
DIPiDIP-59150N
IntActiQ13075, 3 interactors
STRINGi9606.ENSP00000428657

Structurei

Secondary structure

11403
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi159 – 164Combined sources6
Helixi165 – 168Combined sources4
Helixi171 – 173Combined sources3
Helixi178 – 183Combined sources6
Beta strandi186 – 188Combined sources3
Beta strandi195 – 197Combined sources3
Turni198 – 200Combined sources3
Beta strandi203 – 206Combined sources4
Helixi213 – 220Combined sources8
Helixi225 – 231Combined sources7

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2VM5X-ray1.80A141-244[»]
ProteinModelPortaliQ13075
SMRiQ13075
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ13075

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Repeati60 – 127BIR 1Add BLAST68
Repeati159 – 227BIR 2Add BLAST69
Repeati278 – 345BIR 3Add BLAST68
Domaini464 – 758NACHTPROSITE-ProRule annotationAdd BLAST295

Domaini

Both the BIR and NACHT domains are essential for effective inhibition of pro-CASP9 cleavage. BIR3 domain binds to procaspase-9 and the NACHT domain interacts with the NACHT domain of APAF1 forming a bridge between pro-CASP9 and APAF1.1 Publication

Keywords - Domaini

Repeat

Phylogenomic databases

eggNOGiKOG1101 Eukaryota
ENOG410YPNM LUCA
GeneTreeiENSGT00500000044782
HOGENOMiHOG000236326
HOVERGENiHBG050689
InParanoidiQ13075
KOiK12807
OMAiNMLSWLL
OrthoDBiEOG091G0115
PhylomeDBiQ13075
TreeFamiTF105356

Family and domain databases

CDDicd00022 BIR, 3 hits
Gene3Di3.80.10.10, 1 hit
InterProiView protein in InterPro
IPR003593 AAA+_ATPase
IPR001370 BIR_rpt
IPR032675 LRR_dom_sf
IPR007111 NACHT_NTPase
IPR028789 Naip
IPR027417 P-loop_NTPase
PANTHERiPTHR10044:SF134 PTHR10044:SF134, 1 hit
PfamiView protein in Pfam
PF00653 BIR, 3 hits
SMARTiView protein in SMART
SM00382 AAA, 1 hit
SM00238 BIR, 3 hits
SUPFAMiSSF52540 SSF52540, 1 hit
PROSITEiView protein in PROSITE
PS01282 BIR_REPEAT_1, 3 hits
PS50143 BIR_REPEAT_2, 3 hits
PS50837 NACHT, 1 hit

Sequences (2)i

Sequence statusi: Complete.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q13075-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MATQQKASDE RISQFDHNLL PELSALLGLD AVQLAKELEE EEQKERAKMQ
60 70 80 90 100
KGYNSQMRSE AKRLKTFVTY EPYSSWIPQE MAAAGFYFTG VKSGIQCFCC
110 120 130 140 150
SLILFGAGLT RLPIEDHKRF HPDCGFLLNK DVGNIAKYDI RVKNLKSRLR
160 170 180 190 200
GGKMRYQEEE ARLASFRNWP FYVQGISPCV LSEAGFVFTG KQDTVQCFSC
210 220 230 240 250
GGCLGNWEEG DDPWKEHAKW FPKCEFLRSK KSSEEITQYI QSYKGFVDIT
260 270 280 290 300
GEHFVNSWVQ RELPMASAYC NDSIFAYEEL RLDSFKDWPR ESAVGVAALA
310 320 330 340 350
KAGLFYTGIK DIVQCFSCGG CLEKWQEGDD PLDDHTRCFP NCPFLQNMKS
360 370 380 390 400
SAEVTPDLQS RGELCELLET TSESNLEDSI AVGPIVPEMA QGEAQWFQEA
410 420 430 440 450
KNLNEQLRAA YTSASFRHMS LLDISSDLAT DHLLGCDLSI ASKHISKPVQ
460 470 480 490 500
EPLVLPEVFG NLNSVMCVEG EAGSGKTVLL KKIAFLWASG CCPLLNRFQL
510 520 530 540 550
VFYLSLSSTR PDEGLASIIC DQLLEKEGSV TEMCVRNIIQ QLKNQVLFLL
560 570 580 590 600
DDYKEICSIP QVIGKLIQKN HLSRTCLLIA VRTNRARDIR RYLETILEIK
610 620 630 640 650
AFPFYNTVCI LRKLFSHNMT RLRKFMVYFG KNQSLQKIQK TPLFVAAICA
660 670 680 690 700
HWFQYPFDPS FDDVAVFKSY MERLSLRNKA TAEILKATVS SCGELALKGF
710 720 730 740 750
FSCCFEFNDD DLAEAGVDED EDLTMCLMSK FTAQRLRPFY RFLSPAFQEF
760 770 780 790 800
LAGMRLIELL DSDRQEHQDL GLYHLKQINS PMMTVSAYNN FLNYVSSLPS
810 820 830 840 850
TKAGPKIVSH LLHLVDNKES LENISENDDY LKHQPEISLQ MQLLRGLWQI
860 870 880 890 900
CPQAYFSMVS EHLLVLALKT AYQSNTVAAC SPFVLQFLQG RTLTLGALNL
910 920 930 940 950
QYFFDHPESL SLLRSIHFPI RGNKTSPRAH FSVLETCFDK SQVPTIDQDY
960 970 980 990 1000
ASAFEPMNEW ERNLAEKEDN VKSYMDMQRR ASPDLSTGYW KLSPKQYKIP
1010 1020 1030 1040 1050
CLEVDVNDID VVGQDMLEIL MTVFSASQRI ELHLNHSRGF IESIRPALEL
1060 1070 1080 1090 1100
SKASVTKCSI SKLELSAAEQ ELLLTLPSLE SLEVSGTIQS QDQIFPNLDK
1110 1120 1130 1140 1150
FLCLKELSVD LEGNINVFSV IPEEFPNFHH MEKLLIQISA EYDPSKLVKL
1160 1170 1180 1190 1200
IQNSPNLHVF HLKCNFFSDF GSLMTMLVSC KKLTEIKFSD SFFQAVPFVA
1210 1220 1230 1240 1250
SLPNFISLKI LNLEGQQFPD EETSEKFAYI LGSLSNLEEL ILPTGDGIYR
1260 1270 1280 1290 1300
VAKLIIQQCQ QLHCLRVLSF FKTLNDDSVV EIAKVAISGG FQKLENLKLS
1310 1320 1330 1340 1350
INHKITEEGY RNFFQALDNM PNLQELDISR HFTECIKAQA TTVKSLSQCV
1360 1370 1380 1390 1400
LRLPRLIRLN MLSWLLDADD IALLNVMKER HPQSKYLTIL QKWILPFSPI

IQK
Length:1,403
Mass (Da):159,582
Last modified:May 30, 2006 - v3
Checksum:iC1CE163D55900C6D
GO
Isoform 2 (identifier: Q13075-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-61: MATQQKASDE...GYNSQMRSEA → MPLHIGDFVW...IRLELWINLR
     62-223: Missing.

Show »
Length:1,241
Mass (Da):141,329
Checksum:i51648FB32357B99A
GO

Sequence cautioni

The sequence AAC62261 differs from that shown. Reason: Erroneous gene model prediction.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti386 – 387VP → ST in AAA64504 (PubMed:7552146).Curated2
Sequence conflicti553Y → H in AAA64504 (PubMed:7552146).Curated1
Sequence conflicti567 – 568IQ → FK in BAD92360 (Ref. 7) Curated2
Sequence conflicti919P → S in BAD92360 (Ref. 7) Curated1
Sequence conflicti1066S → T in BAD92360 (Ref. 7) Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_026477535V → M5 Publications1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0471961 – 61MATQQ…MRSEA → MPLHIGDFVWDSKVHSLQSS LNIFSLLPTKGRTEHLFFSH ILSFHWPAFSSIRLELWINL R in isoform 2. 1 PublicationAdd BLAST61
Alternative sequenceiVSP_04719762 – 223Missing in isoform 2. 1 PublicationAdd BLAST162

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U19251 mRNA Translation: AAC52045.1
U80017 Genomic DNA Translation: AAC52047.1
AC005031 Genomic DNA Translation: AAC62261.1 Sequence problems.
AC044797 Genomic DNA No translation available.
BC136273 mRNA Translation: AAI36274.1
AB048534 mRNA Translation: BAB87181.1
AH003063 mRNA Translation: AAA64504.1
AB209123 mRNA Translation: BAD92360.1
CCDSiCCDS4009.1 [Q13075-1]
CCDS43327.1 [Q13075-2]
RefSeqiNP_001333799.1, NM_001346870.1 [Q13075-1]
NP_004527.2, NM_004536.2 [Q13075-1]
NP_075043.1, NM_022892.1 [Q13075-2]
UniGeneiHs.646951
Hs.654500
Hs.710305

Genome annotation databases

EnsembliENST00000194097; ENSP00000443944; ENSG00000249437 [Q13075-1]
ENST00000503719; ENSP00000424913; ENSG00000249437 [Q13075-2]
ENST00000517649; ENSP00000428657; ENSG00000249437 [Q13075-1]
ENST00000523981; ENSP00000428363; ENSG00000249437 [Q13075-2]
ENST00000612328; ENSP00000484107; ENSG00000278613
ENST00000620988; ENSP00000484731; ENSG00000278613
GeneIDi4671
KEGGihsa:4671
UCSCiuc003kar.2 human [Q13075-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Entry informationi

Entry nameiBIRC1_HUMAN
AccessioniPrimary (citable) accession number: Q13075
Secondary accession number(s): B9EG72
, E9PHD1, O75857, Q13730, Q59GI6, Q8TDZ4, Q99796
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: May 30, 2006
Last modified: May 23, 2018
This is version 165 of the entry and version 3 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human chromosome 5
    Human chromosome 5: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references

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