Atlas of Genetics and Cytogenetics in Oncology and Haematology


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S100B (S100 calcium binding protein B)

Identity

Other namesNEF
S100
S100Beta
Hugo S100B
Location 21q22.3

DNA/RNA

Description The gene encompasses 17.3 Kb of DNA; 3 exons (the first one contains the 5' untranslated region).
Transcription 1135 b mRNA; 279 b coding sequence.

Protein

Description 92 amino acids (including initial methionine that is generally processed in vivo);
10.5 kDa monomer (S100B can form homodimers and heterodimers with other proteins of the S100 family, described for S100A1).
Expression S100B highest levels are found in brain. The protein is primarily found in astrocytes. Outside the central nervous system it can be found in chondrocytes and melanocytes.
Localisation Nuclear and cytoplasmic. It has both intracellular and extracellular roles.
Function The exact function of S100B is not fully understood. It inhibits microtubule assembly, has been involved in the regulation of cell cycle progression and differentiation and is able to induce neurite extension. This latest effect seems to be dependent on the concentration of S100B and occurs at nanomolar concentrations.
But micromolar levels of extracellular S100B stimulate apoptosis in vitro. Calcium binding induces a conformational change in S100B that allows the interaction with a variety of target proteins. These include p53 tumour suppressor, the microtubule-associated protein tau, the cytoskeletal protein tubulin (and its prokaryotic ancestor FtsZ), the scaffold protein IQGAP1, the intermediate filament protein glial fibrillary acidic protein (GFAP),the actin capping protein CapZ and the giant phosphoprotein AHNAK. Over-expression of S100B has been proposed to play a role in different neuro-pathologies.
Homology S100B belongs to the S100 family of calcium binding proteins, a highly homologous family. These proteins contain two EF-hand calcium binding domains. The S100 genes are present exclusively in vertebrates.

Mutations

Note Have not been reported.

Implicated in

Disease Overexpression of S100B has been generally linked to neurodegeneration. It is over-expressed in the brain of patients suffering from Alzheimer's disease, epilepsy or amyotrophic lateral sclerosis. The gene coding for S100B maps in the Down's syndrome region of chromosome 21, and its over-expression, due to the trisomic state, may be responsible for the neurological disturbances in Down's syndrome.
Oncogenesis S100B may be involved in the proliferation of melanoma cells. It has been shown to be elevated in primary malignant melanomas. However, S100B is used as a predictor of survival prognosis as elevated levels of S100B in serum are associated with the survival rate.
  

External links

Nomenclature
HugoS100B
GDBS100B
Entrez_GeneS100B  6285  S100 calcium binding protein B
Cards
AtlasS100BID42195ch21q22
GeneCardsS100B
EnsemblS100B [Search_View]   ENSG00000160307 [Gene_View]
GenatlasS100B
GeneLynxS100B
eGenomeS100B
euGene6285
Genomic and cartography
GoldenPathS100B  -  21q22.3   chr21:46842959-46849463 -  21q22.3   [Description]    (hg18-Mar_2006)
EnsemblS100B - 21q22.3 [CytoView]
NCBIMapview
OMIMDisease map [OMIM]
HomoloGeneS100B
Gene and transcription
GenbankAA310307 [ ENTREZ ]
GenbankBC001766 [ ENTREZ ]
GenbankBC041935 [ ENTREZ ]
GenbankCR542123 [ ENTREZ ]
GenbankDQ892491 [ ENTREZ ]
RefSeqNM_006272 [ SRS ]    NM_006272 [ ENTREZ ]
RefSeqAC_000064 [ SRS ]    AC_000064 [ ENTREZ ]
RefSeqNC_000021 [ SRS ]    NC_000021 [ ENTREZ ]
RefSeqNT_011515 [ SRS ]    NT_011515 [ ENTREZ ]
RefSeqNW_927384 [ SRS ]    NW_927384 [ ENTREZ ]
AceViewS100B AceView - NCBI
UnigeneHs.422181 [ SRS ]    Hs.422181 [ NCBI ]     HS422181 [ spliceNest ]
Fast-db5009 (alternative variants)
Protein : pattern, domain, 3D structure
SwissProtP04271 [ SRS]    P04271 [ EXPASY ]     P04271 [ INTERPRO ]
PrositePS00018 EF_HAND_1 [ SRS ]    PS00018 EF_HAND_1 [ Expasy ]
PrositePS50222 EF_HAND_2 [ SRS ]    PS50222 EF_HAND_2 [ Expasy ]
PrositePS00303 S100_CABP [ SRS ]    PS00303 S100_CABP [ Expasy ]
InterproIPR011992 EF-Hand_type [ SRS ]    IPR011992 EF-Hand_type [ EBI ]
InterproIPR002048 EF_hand_Ca_bd [ SRS ]    IPR002048 EF_hand_Ca_bd [ EBI ]
InterproIPR001751 S100_Ca_bd [ SRS ]    IPR001751 S100_Ca_bd [ EBI ]
InterproIPR013787 S100_Ca_bd_sub [ SRS ]    IPR013787 S100_Ca_bd_sub [ EBI ]
CluSTrP04271
PfamPF00036 efhand [ SRS ]    PF00036 efhand [ Sanger ]    pfam00036 [ NCBI-CDD ]
PfamPF01023 S_100 [ SRS ]    PF01023 S_100 [ Sanger ]    pfam01023 [ NCBI-CDD ]
ProdomPD003407 CaBP_S100[INRA-Toulouse]
ProdomP04271 S100B_HUMAN [ Domain structure ]   P04271 S100B_HUMAN  [ sequences sharing at least 1 domain ]
ProdomPD003407[INRA-Toulouse]
ProdomP04271 S100B_HUMAN [ Domain structure ]   P04271 S100B_HUMAN  [ sequences sharing at least 1 domain ]
BlocksP04271
PDB1MQ1 [ SRS ]    1MQ1 [ PdbSum ],   1MQ1 [ IMB ]   1MQ1 [ RSDB ]
PDB1UWO [ SRS ]    1UWO [ PdbSum ],   1UWO [ IMB ]   1UWO [ RSDB ]
PDB2H61 [ SRS ]    2H61 [ PdbSum ],   2H61 [ IMB ]   2H61 [ RSDB ]
HPRD01505
Protein Interaction databases
DIPP04271
IntActP04271
Polymorphism : SNP, mutations, diseases
OMIM176990    [ map ]   
GENECLINICS176990
SNPS100B [dbSNP-NCBI]  
SNPNM_006272 [SNP-NCI]  
SNPS100B [GeneSNPs - Utah]  S100B] [HGBASE - SRS]
HAPMAPS100B [HAPMAP]  
HGMDS100B
General knowledge
Family BrowserS100B [UCSC Family Browser]
SOURCENM_006272
SMDHs.422181
SAGEHs.422181
GOruffle [Amigo]  ruffle
GOcalcium ion binding [Amigo]  calcium ion binding
GOnucleus [Amigo]  nucleus
GOcytoplasm [Amigo]  cytoplasm
GOcytoplasm [Amigo]  cytoplasm
GOaxonogenesis [Amigo]  axonogenesis
GOcentral nervous system development [Amigo]  central nervous system development
GOlearning and/or memory [Amigo]  learning and/or memory
GOzinc ion binding [Amigo]  zinc ion binding
GOcell proliferation [Amigo]  cell proliferation
GOprotein homodimerization activity [Amigo]  protein homodimerization activity
GOprotein homodimerization activity [Amigo]  protein homodimerization activity
GOS100 beta binding [Amigo]  S100 beta binding
GOtau protein binding [Amigo]  tau protein binding
GOcalcium-dependent protein binding [Amigo]  calcium-dependent protein binding
PubGeneS100B
TreeFamS100B
CTD6285 [Comparative ToxicoGenomics Database]
Other databases
Probes
ProbeS100B Related clones (RZPD - Berlin)
PubMed
PubMed91 Pubmed reference(s) in LocusLink

Bibliography

Calcium-dependent interaction of S100b, troponin C, and calmodulin with an immobilized phenothiazine.
Marshak DR, Watterson DM, Van Eldik LJ
Proceedings of the National Academy of Sciences of the United States of America. 1981 ; 78 (11) : 6793-6797.
PMID 6947252
 
Ions binding to S100 proteins: structural changes induced by calcium and zinc on S100a and S100b proteins.
Baudier J, Gˆ©rard D
Biochemistry. 1983 ; 22 (14) : 3360-3369.
PMID 6615778
 
Comparison of S100b protein with calmodulin: interactions with melittin and microtubule-associated tau proteins and inhibition of phosphorylation of tau proteins by protein kinase C.
Baudier J, Mochly-Rosen D, Newton A, Lee SH, Koshland DE Jr, Cole RD
Biochemistry. 1987 ; 26 (10) : 2886-2893.
PMID 3111527
 
Gene encoding the beta subunit of S100 protein is on chromosome 21: implications for Down syndrome.
Allore R, O'Hanlon D, Price R, Neilson K, Willard HF, Cox DR, Marks A, Dunn RJ
Science (New York, N.Y.). 1988 ; 239 (4845) : 1311-1313.
PMID 2964086
 
Interactions between the microtubule-associated tau proteins and S100b regulate tau phosphorylation by the Ca2+/calmodulin-dependent protein kinase II.
Baudier J, Cole RD
The Journal of biological chemistry. 1988 ; 263 (12) : 5876-5883.
PMID 2833519
 
The Ca2+-binding sequence in bovine brain S100b protein beta-subunit. A spectroscopic study.
Baudier J, Cole RD
The Biochemical journal. 1989 ; 264 (1) : 79-85.
PMID 2604719
 
Characterization of the tumor suppressor protein p53 as a protein kinase C substrate and a S100b-binding protein.
Baudier J, Delphin C, Grunwald D, Khochbin S, Lawrence JJ
Proceedings of the National Academy of Sciences of the United States of America. 1992 ; 89 (23) : 11627-11631.
PMID 1454855
 
Astrocytosis and axonal proliferation in the hippocampus of S100b transgenic mice.
Reeves RH, Yao J, Crowley MR, Buck S, Zhang X, Yarowsky P, Gearhart JD, Hilt DC
Proceedings of the National Academy of Sciences of the United States of America. 1994 ; 91 (12) : 5359-5363.
PMID 8202493
 
Solution structure of rat apo-S100B(beta beta) as determined by NMR spectroscopy.
Drohat AC, Amburgey JC, Abildgaard F, Starich MR, Baldisseri D, Weber DJ
Biochemistry. 1996 ; 35 (36) : 11577-11588.
PMID 8794737
 
The solution structure of the bovine S100B protein dimer in the calcium-free state.
Kilby PM, Van Eldik LJ, Roberts GC
Structure (London, England : 1993). 1996 ; 4 (9) : 1041-1052.
PMID 8805590
 
S100B protein, 5-S-cysteinyldopa and 6-hydroxy-5-methoxyindole-2-carboxylic acid as biochemical markers for survival prognosis in patients with malignant melanoma.
Kˆ§rnell R, von Schoultz E, Hansson LO, Nilsson B, Arstrand K, Kˆ€gedal B
Melanoma research. 1997 ; 7 (5) : 393-399.
PMID 9429222
 
Identification of the binding site on S100B protein for the actin capping protein CapZ.
Kilby PM, Van Eldik LJ, Roberts GC
Protein science : a publication of the Protein Society. 1997 ; 6 (12) : 2494-2503.
PMID 9416599
 
Solution structure of calcium-bound rat S100B(betabeta) as determined by nuclear magnetic resonance spectroscopy,.
Drohat AC, Baldisseri DM, Rustandi RR, Weber DJ
Biochemistry. 1998 ; 37 (9) : 2729-2740.
PMID 9485423
 
Calcium regulation of Ndr protein kinase mediated by S100 calcium-binding proteins.
Millward TA, Heizmann CW, Schˆ§fer BW, Hemmings BA
The EMBO journal. 1998 ; 17 (20) : 5913-5922.
PMID 9774336
 
The Ca(2+)-dependent interaction of S100B(beta beta) with a peptide derived from p53.
Rustandi RR, Drohat AC, Baldisseri DM, Wilder PT, Weber DJ
Biochemistry. 1998 ; 37 (7) : 1951-1960.
PMID 9485322
 
Calcium and S100B regulation of p53-dependent cell growth arrest and apoptosis.
Scotto C, Deloulme JC, Rousseau D, Chambaz E, Baudier J
Molecular and cellular biology. 1998 ; 18 (7) : 4272-4281.
PMID 9632811
 
Association of S100B with intermediate filaments and microtubules in glial cells.
Sorci G, Agneletti AL, Bianchi R, Donato R
Biochimica et biophysica acta. 1998 ; 1448 (2) : 277-289.
PMID 9920418
 
Calcium-dependent interaction of S100B with the C-terminal domain of the tumor suppressor p53.
Delphin C, Ronjat M, Deloulme JC, Garin G, Debussche L, Higashimoto Y, Sakaguchi K, Baudier J
The Journal of biological chemistry. 1999 ; 274 (15) : 10539-10544.
PMID 10187847
 
Concerted regulation of wild-type p53 nuclear accumulation and activation by S100B and calcium-dependent protein kinase C.
Scotto C, Delphin C, Deloulme JC, Baudier J
Molecular and cellular biology. 1999 ; 19 (10) : 7168-7180.
PMID 10490652
 
Structure of the negative regulatory domain of p53 bound to S100B(betabeta).
Rustandi RR, Baldisseri DM, Weber DJ
Nature structural biology. 2000 ; 7 (7) : 570-574.
PMID 10876243
 
Glial protein S100B modulates long-term neuronal synaptic plasticity.
Nishiyama H, Knopfel T, Endo S, Itohara S
Proceedings of the National Academy of Sciences of the United States of America. 2002 ; 99 (6) : 4037-4042.
PMID 11891290
 
S100B inhibits myogenic differentiation and myotube formation in a RAGE-independent manner.
Sorci G, Riuzzi F, Agneletti AL, Marchetti C, Donato R
Molecular and cellular biology. 2003 ; 23 (14) : 4870-4881.
PMID 12832473
 
Proteins of the S100 family regulate the oligomerization of p53 tumor suppressor.
Fernandez-Fernandez MR, Veprintsev DB, Fersht AR
Proceedings of the National Academy of Sciences of the United States of America. 2005 ; 102 (13) : 4735-4740.
PMID 15781852
 
S100B-induced microglial and neuronal IL-1 expression is mediated by cell type-specific transcription factors.
Liu L, Li Y, Van Eldik LJ, Griffin WS, Barger SW
Journal of neurochemistry. 2005 ; 92 (3) : 546-553.
PMID 15659225
 
Key role of Src kinase in S100B-induced activation of the receptor for advanced glycation end products in vascular smooth muscle cells.
Reddy MA, Li SL, Sahar S, Kim YS, Xu ZG, Lanting L, Natarajan R
The Journal of biological chemistry. 2006 ; 281 (19) : 13685-13693.
PMID 16551628
 
Expression of S100B during embryonic development of the mouse cerebellum.
Hachem S, Laurenson AS, Hugnot JP, Legraverend C
BMC developmental biology. 2007 ; 7 : page 17.
PMID 17362503
 
Evidence for a wide extra-astrocytic distribution of S100B in human brain.
Steiner J, Bernstein HG, Bielau H, Berndt A, Brisch R, Mawrin C, Keilhoff G, Bogerts B
BMC neuroscience. 2007 ; 8 : page 2.
PMID 17199889
 
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Contributor(s)

Written05-2007M Rosario Fernandez-Fernandez, Alan R Fersht
Centre for Protein Engineering, Medical Research Council, Hills Road, Cambridge CB2 2QH, United Kingdom

Citation

This paper should be referenced as such :
Fernandez-Fernandez MR, Fersht AR . S100B (S100 calcium binding protein B). Atlas Genet Cytogenet Oncol Haematol. May 2007 .
URL : http://AtlasGeneticsOncology.org/Genes/S100BID42195ch21q22.html

© Atlas of Genetics and Cytogenetics in Oncology and Haematology
indexed on : Wed Jul 2 08:26:56 2008


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