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Q99574

- NEUS_HUMAN

UniProt

Q99574 - NEUS_HUMAN

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Protein
Neuroserpin
Gene
SERPINI1, PI12
Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5 - Experimental evidence at protein leveli

Functioni

Serine protease inhibitor that inhibits plasminogen activators and plasmin but not thrombin. May be involved in the formation or reorganization of synaptic connections as well as for synaptic plasticity in the adult nervous system. May protect neurons from cell damage by tissue-type plasminogen activator.

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sitei362 – 3632Reactive bond By similarity

GO - Molecular functioni

  1. serine-type endopeptidase inhibitor activity Source: RefGenome

GO - Biological processi

  1. cell death Source: UniProtKB-KW
  2. central nervous system development Source: ProtInc
  3. negative regulation of endopeptidase activity Source: RefGenome
  4. peripheral nervous system development Source: ProtInc
  5. regulation of cell adhesion Source: Ensembl
  6. regulation of proteolysis Source: RefGenome
Complete GO annotation...

Keywords - Molecular functioni

Protease inhibitor, Serine protease inhibitor

Protein family/group databases

MEROPSiI04.025.

Names & Taxonomyi

Protein namesi
Recommended name:
Neuroserpin
Alternative name(s):
Peptidase inhibitor 12
Short name:
PI-12
Serpin I1
Gene namesi
Synonyms:PI12
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640: Chromosome 3

Organism-specific databases

HGNCiHGNC:8943. SERPINI1.

Subcellular locationi

GO - Cellular componenti

  1. extracellular space Source: InterPro
  2. extracellular vesicular exosome Source: UniProt
Complete GO annotation...

Keywords - Cellular componenti

Secreted

Pathology & Biotechi

Involvement in diseasei

Encephalopathy, familial, with neuroserpin inclusion bodies (FENIB) [MIM:604218]: A neurodegenerative disease clinically characterized by dementia. Additional features include intellectual decline, psychic seizures, progressive myoclonic epilepsy, and cerebral atrophy. Histologically, it is characterized by the presence of eosinophilic inclusion bodies (called Collins bodies) throughout the deeper layers of the cerebral cortex, leading to neuronal death.
Note: The disease is caused by mutations affecting the gene represented in this entry.1 Publication
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti49 – 491S → P in FENIB; Syracuse. 1 Publication
VAR_008520
Natural varianti52 – 521S → R in FENIB; Portland. 1 Publication
VAR_008521

Keywords - Diseasei

Disease mutation, Neurodegeneration

Organism-specific databases

MIMi604218. phenotype.
Orphaneti85110. Familial encephalopathy with neuroserpin inclusion bodies.
PharmGKBiPA35511.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Signal peptidei1 – 1616 Reviewed prediction
Add
BLAST
Chaini17 – 410394Neuroserpin
PRO_0000032521Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Glycosylationi157 – 1571N-linked (GlcNAc...) Reviewed prediction
Glycosylationi321 – 3211N-linked (GlcNAc...) Reviewed prediction
Glycosylationi401 – 4011N-linked (GlcNAc...) Reviewed prediction

Keywords - PTMi

Glycoprotein

Proteomic databases

MaxQBiQ99574.
PaxDbiQ99574.
PRIDEiQ99574.

PTM databases

PhosphoSiteiQ99574.

Miscellaneous databases

PMAP-CutDBQ99574.

Expressioni

Tissue specificityi

Predominantly expressed in the brain.

Gene expression databases

ArrayExpressiQ99574.
BgeeiQ99574.
CleanExiHS_SERPINI1.
GenevestigatoriQ99574.

Organism-specific databases

HPAiCAB026059.
HPA001565.

Interactioni

Protein-protein interaction databases

BioGridi111292. 2 interactions.
IntActiQ99574. 1 interaction.
STRINGi9606.ENSP00000295777.

Structurei

Secondary structure

Legend: HelixTurnBeta strand
Show more details
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Helixi25 – 4016
Beta strandi46 – 483
Helixi50 – 6314
Helixi66 – 7611
Helixi86 – 916
Turni101 – 1033
Beta strandi105 – 11511
Helixi122 – 13211
Beta strandi135 – 1395
Helixi144 – 15714
Turni158 – 1614
Helixi169 – 1713
Beta strandi176 – 19217
Helixi196 – 1983
Beta strandi200 – 2056
Beta strandi211 – 22919
Beta strandi238 – 2469
Beta strandi249 – 2579
Helixi264 – 2674
Helixi268 – 2703
Helixi273 – 28210
Beta strandi284 – 29310
Beta strandi295 – 3028
Helixi303 – 3108
Helixi314 – 3163
Turni323 – 3253
Beta strandi331 – 34414
Beta strandi346 – 36116
Beta strandi369 – 3713
Beta strandi376 – 3827
Turni383 – 3853
Beta strandi388 – 3958

3D structure databases

Select the link destinations:
PDBe
RCSB PDB
PDBj
Links Updated
EntryMethodResolution (Å)ChainPositionsPDBsum
3F02X-ray1.80A/B17-362[»]
C/D363-410[»]
3F5NX-ray3.15A/B/C/D/E17-410[»]
3FGQX-ray2.09A/B17-400[»]
ProteinModelPortaliQ99574.
SMRiQ99574. Positions 22-400.

Miscellaneous databases

EvolutionaryTraceiQ99574.

Family & Domainsi

Sequence similaritiesi

Belongs to the serpin family.

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiCOG4826.
HOGENOMiHOG000238519.
HOVERGENiHBG005957.
InParanoidiQ99574.
OMAiMMKKYFN.
PhylomeDBiQ99574.
TreeFamiTF352620.

Family and domain databases

InterProiIPR023795. Serpin_CS.
IPR023796. Serpin_dom.
IPR000215. Serpin_fam.
[Graphical view]
PANTHERiPTHR11461. PTHR11461. 1 hit.
PfamiPF00079. Serpin. 1 hit.
[Graphical view]
SMARTiSM00093. SERPIN. 1 hit.
[Graphical view]
SUPFAMiSSF56574. SSF56574. 1 hit.
PROSITEiPS00284. SERPIN. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

Q99574-1 [UniParc]FASTAAdd to Basket

« Hide

MAFLGLFSLL VLQSMATGAT FPEEAIADLS VNMYNRLRAT GEDENILFSP    50
LSIALAMGMM ELGAQGSTQK EIRHSMGYDS LKNGEEFSFL KEFSNMVTAK 100
ESQYVMKIAN SLFVQNGFHV NEEFLQMMKK YFNAAVNHVD FSQNVAVANY 150
INKWVENNTN NLVKDLVSPR DFDAATYLAL INAVYFKGNW KSQFRPENTR 200
TFSFTKDDES EVQIPMMYQQ GEFYYGEFSD GSNEAGGIYQ VLEIPYEGDE 250
ISMMLVLSRQ EVPLATLEPL VKAQLVEEWA NSVKKQKVEV YLPRFTVEQE 300
IDLKDVLKAL GITEIFIKDA NLTGLSDNKE IFLSKAIHKS FLEVNEEGSE 350
AAAVSGMIAI SRMAVLYPQV IVDHPFFFLI RNRRTGTILF MGRVMHPETM 400
NTSGHDFEEL 410
Length:410
Mass (Da):46,427
Last modified:May 1, 1997 - v1
Checksum:iD966E9036BB21943
GO

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti49 – 491S → P in FENIB; Syracuse. 1 Publication
VAR_008520
Natural varianti52 – 521S → R in FENIB; Portland. 1 Publication
VAR_008521

Sequence conflict

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti70 – 701K → E in AAH18043. 1 Publication
Sequence conflicti326 – 3261S → Y in AAH18043. 1 Publication

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
Z81326 mRNA. Translation: CAB03626.1.
AF248246, AF248244, AF248245 Genomic DNA. Translation: AAG01089.1.
AK290082 mRNA. Translation: BAF82771.1.
CR627434 mRNA. Translation: CAH10520.1.
CH471052 Genomic DNA. Translation: EAW78571.1.
CH471052 Genomic DNA. Translation: EAW78572.1.
CH471052 Genomic DNA. Translation: EAW78573.1.
BC018043 mRNA. Translation: AAH18043.1.
CCDSiCCDS3203.1.
RefSeqiNP_001116224.1. NM_001122752.1.
NP_005016.1. NM_005025.4.
XP_006713720.1. XM_006713657.1.
UniGeneiHs.478153.

Genome annotation databases

EnsembliENST00000295777; ENSP00000295777; ENSG00000163536.
ENST00000446050; ENSP00000397373; ENSG00000163536.
GeneIDi5274.
KEGGihsa:5274.
UCSCiuc003ffa.4. human.

Polymorphism databases

DMDMi3183087.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
Z81326 mRNA. Translation: CAB03626.1 .
AF248246 , AF248244 , AF248245 Genomic DNA. Translation: AAG01089.1 .
AK290082 mRNA. Translation: BAF82771.1 .
CR627434 mRNA. Translation: CAH10520.1 .
CH471052 Genomic DNA. Translation: EAW78571.1 .
CH471052 Genomic DNA. Translation: EAW78572.1 .
CH471052 Genomic DNA. Translation: EAW78573.1 .
BC018043 mRNA. Translation: AAH18043.1 .
CCDSi CCDS3203.1.
RefSeqi NP_001116224.1. NM_001122752.1.
NP_005016.1. NM_005025.4.
XP_006713720.1. XM_006713657.1.
UniGenei Hs.478153.

3D structure databases

Select the link destinations:
PDBe
RCSB PDB
PDBj
Links Updated
Entry Method Resolution (Å) Chain Positions PDBsum
3F02 X-ray 1.80 A/B 17-362 [» ]
C/D 363-410 [» ]
3F5N X-ray 3.15 A/B/C/D/E 17-410 [» ]
3FGQ X-ray 2.09 A/B 17-400 [» ]
ProteinModelPortali Q99574.
SMRi Q99574. Positions 22-400.
ModBasei Search...
MobiDBi Search...

Protein-protein interaction databases

BioGridi 111292. 2 interactions.
IntActi Q99574. 1 interaction.
STRINGi 9606.ENSP00000295777.

Protein family/group databases

MEROPSi I04.025.

PTM databases

PhosphoSitei Q99574.

Polymorphism databases

DMDMi 3183087.

Proteomic databases

MaxQBi Q99574.
PaxDbi Q99574.
PRIDEi Q99574.

Protocols and materials databases

DNASUi 5274.
Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENST00000295777 ; ENSP00000295777 ; ENSG00000163536 .
ENST00000446050 ; ENSP00000397373 ; ENSG00000163536 .
GeneIDi 5274.
KEGGi hsa:5274.
UCSCi uc003ffa.4. human.

Organism-specific databases

CTDi 5274.
GeneCardsi GC03P167453.
HGNCi HGNC:8943. SERPINI1.
HPAi CAB026059.
HPA001565.
MIMi 602445. gene.
604218. phenotype.
neXtProti NX_Q99574.
Orphaneti 85110. Familial encephalopathy with neuroserpin inclusion bodies.
PharmGKBi PA35511.
GenAtlasi Search...

Phylogenomic databases

eggNOGi COG4826.
HOGENOMi HOG000238519.
HOVERGENi HBG005957.
InParanoidi Q99574.
OMAi MMKKYFN.
PhylomeDBi Q99574.
TreeFami TF352620.

Miscellaneous databases

EvolutionaryTracei Q99574.
GeneWikii SERPINI1.
GenomeRNAii 5274.
NextBioi 20380.
PMAP-CutDB Q99574.
PROi Q99574.
SOURCEi Search...

Gene expression databases

ArrayExpressi Q99574.
Bgeei Q99574.
CleanExi HS_SERPINI1.
Genevestigatori Q99574.

Family and domain databases

InterProi IPR023795. Serpin_CS.
IPR023796. Serpin_dom.
IPR000215. Serpin_fam.
[Graphical view ]
PANTHERi PTHR11461. PTHR11461. 1 hit.
Pfami PF00079. Serpin. 1 hit.
[Graphical view ]
SMARTi SM00093. SERPIN. 1 hit.
[Graphical view ]
SUPFAMi SSF56574. SSF56574. 1 hit.
PROSITEi PS00284. SERPIN. 1 hit.
[Graphical view ]
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  1. "Human neuroserpin (PI12): cDNA cloning and chromosomal localization to 3q26."
    Schrimpf S.P., Bleiker A.J., Brecevic L., Kozlov S.V., Berger P., Osterwalder T., Krueger S.R., Schinzel A., Sonderegger P.
    Genomics 40:55-62(1997) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
    Tissue: Retina.
  2. "Genomic organization of the human neuroserpin (PI12) gene."
    Kinter J., Berger P., Kozlov S.V., Sonderegger P.
    Submitted (MAR-2000) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA].
  3. "Complete sequencing and characterization of 21,243 full-length human cDNAs."
    Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S.
    , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.
    Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Tissue: Subthalamic nucleus.
  4. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Tissue: Brain cortex.
  5. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  6. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Tissue: Brain.
  7. Cited for: VARIANTS FENIB PRO-49 AND ARG-52.

Entry informationi

Entry nameiNEUS_HUMAN
AccessioniPrimary (citable) accession number: Q99574
Secondary accession number(s): A8K217, D3DNP1, Q6AHZ4
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 15, 1998
Last sequence update: May 1, 1997
Last modified: September 3, 2014
This is version 129 of the entry and version 1 of the sequence. [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 3
    Human chromosome 3: 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
  6. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

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