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Symbol S100B contributors: mct - updated : 24-04-2013
HGNC name S100 calcium binding protein B
HGNC id 10500
Location 21q22.3      Physical location : 48.018.530 - 48.025.035
Synonym name
  • S-100 calcium-binding protein, beta chain
  • S100 calcium binding protein, beta (neural)
  • S100 beta
  • Synonym symbol(s) NEF, S100, S100beta
    TYPE functioning gene
    STRUCTURE 6.51 kb     3 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    text structure probably a locus control region LCR-S100B
    MAPPING cloned Y linked N status confirmed
    Map see COL6A2 , PFKL PFKL
    Physical map
    LOC388830 21 similar to PRED59 COL18A1 21q22.3 collagen, type XVIII, alpha 1 LOC388831 21 LOC388831 SLC19A1 21q22.3 solute carrier family 19 (folate transporter), member 1 LOC388832 21 hypothetical gene supported by BC020585 LOC388833 21 LOC388833 PCBP3 21q22.3 poly(rC) binding protein 3 COL6A1 21q22.3 collagen, type VI, alpha 1 LOC200292 21q22.3 hypothetical LOC200292 COL6A2 21q22.3 collagen, type VI, alpha 2 FTCD 21q22.3 formiminotransferase cyclodeaminase C21orf56 21q22.3 chromosome 21 open reading frame 56 LSS 21q22.3 lanosterol synthase (2,3-oxidosqualene-lanosterol cyclase) MCM3APAS 21q22.3 MCM3 minichromosome maintenance deficient 3 (S. cerevisiae) associated protein, antisense C21orf85 21q22.1 chromosome 21 open reading frame 85 MCM3AP 21q22.3 MCM3 minichromosome maintenance deficient 3 (S. cerevisiae) associated protein C21orf57 21q22.3 chromosome 21 open reading frame 57 C21orf58 21q22.3 chromosome 21 open reading frame 58 PCNT2 21q22.3 pericentrin 2 (kendrin) LOC388834 21 hypothetical gene supported by AK127572 C21orf106 21q22.3 chromosome 21 open reading frame 106 S100B 21q22.3 S100 calcium binding protein, beta (neural) HRMT1L1 21q22.3 HMT1 hnRNP methyltransferase-like 1 (S. cerevisiae) RPL23AP4 21q22.3 ribosomal protein L23a pseudogene 4
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    3 - 1135 10.6 92 - 2008 17984171
    Type widely
       expressed in (based on citations)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Hearing/Equilibriumear   highly
    Nervousbrain   highly Homo sapiens
    Reproductivefemale systembreastmammary gland  
    Skin/Tegumentskin   highly
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Connectivecartilage    Homo sapiens
    SystemCellPubmedSpeciesStageRna symbol
    Nervousastrocyte Homo sapiens
    Nervousoligodendrocyte Homo sapiens
    cell lineage
    cell lines
    fluid/secretion in human milk
    at STAGE
  • two EF-hand calcium-binding motifs
  • mono polymer homomer , dimer
  • S-100 family
  • S-101 family
  • CATEGORY regulatory
    basic FUNCTION
  • stimulating GUCY2D at micromolar concentration of Ca2+ for retinal specific activity
  • S100B contributes to OPC (oligodendrocyte progenitor cell) differentiation in response to demyelinating insult
  • involved in the regulation of a number of cellular processes such as cell cycle progression and differentiation
  • may play a role in the phosphorylation state of cytoskeletal proteins
  • promote beta amyloid precursor protein overexpression
  • can contribute to neurofibrillary tangles formation in Alzheimer disease and in all other conditions in which neuroinflammation may have a crucial role
  • calcium-binding protein with both extracellular and intracellular regulatory activities in the brain
  • EF-hand protein that undergo a calcium-induced conformational change allowing them to interact with other proteins and produce a biological response
  • possible function of S100B in assisting the cytoplasmic processing of proteins for proper folding and subcellular localization
  • plays an important role in modulation and differentiation of neuronal and glial cells
  • likely contributes to cancer progression by down-regulating the tumor suppressor protein, TP53
  • can function as an alternative Ca2+ sensor for the potassium channel
  • reduces myoblast apoptosis in an MEK-ERK1/2, AKT1, JNK, and NFKB-dependent manner
  • might act an unconventional cytokine or a damage-associated molecular pattern protein playing a role in the pathophysiology of neurodegenerative disorders and inflammatory brain diseases
  • might participate in the pathophysiology of brain inflammatory disorders via RAGE-dependent regulation of several inflammation-related events including activation and migration of microglia
  • exerts pro-survival or pro-apoptotic effects depending on the concentration reached in the extracellular millieu (
  • alters neuronal survival and dendrite extension via AGER-mediated NFKB signaling
  • stimulates myoblast proliferation and inhibits myogenic differentiation by activating FGFR1 in a bFGF-dependent manner
  • dimeric EF-hand protein that undergoes a calcium-induced conformational change and exposes a hydrophobic protein-binding surface
  • association of S100A1, and to a lesser extent S100B, with the human articular chondrocytes (HAC) differentiated phenotype
  • S100A1 and S100B are dispensable for endochondral ossification during skeletal development, most likely because their deficiency may be masked by other S100 proteins which have similar functions
    a component
  • dimer of either two alpha chains, or two beta chains, or one alpha and one beta chain
  • S100B formed a complex with TP53 in the presence of MDM2
    small molecule
  • STK38
  • interacting with a variety of target proteins, including the cytoskeletal proteins tubulin and glial fibrillary acidic protein
  • interacts with the tubulin homologue FtsZ (E.Coli) modulating its activity in bacterial cell division
  • interacting with DRD2 (enhances receptor signaling)
  • transcriptional target of the SOX trio (SOX5, SOX6, SOX9) and mediate its inhibition of terminal differentiation of chondrocytes
  • binding to the TP53 tetramerization domain (
  • S100B-ATAD3A interaction could significantly increase the calcium affinity by decreasing the off rate of calcium, which was observed previously for S100B and for other S100-target protein interactions
  • interaction between S100B and the N-terminal domain of MDM2, dependent on calcium
  • binds to KCNH1 and shares the same binding sites with Calmodulin
  • S100B receptor is AGER
  • binding of S100B to AGER results in stimulation of AGER anti-mitogenic and promyogenic signaling
  • binding partner of the dopamine D2 receptor
  • S100A1, S100A2, S100A6, and S100B proteins specifically interact with PPP5C-tetratricopeptide repeat (TPR) domain
  • cell & other
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    chromosomal rearrangements implicated in several neurological, neoplastic, Alzheimer's disease, Down's syndrome, epilepsy, amyotrophic lateral sclerosis, melanoma, and type I diabetes
    constitutional     --over  
    in the amnion of pre-eclamptic patients and patients with pre-eclampsia with IUGR
    tumoral     --over  
    found in malignant melanoma contribute to cancer progression by down-regulating TP53 activity
    tumoral     --over  
    in malignant melanoma and to a lesser extent in thyroid carcinoma and renal cell carcinoma
    constitutional     --over  
    associated with the severity of cardiac dysfunction, renal insufficiency (RI) and an adverse prognosis in chronic heart failure (CHF) patients
    constitutional     --over  
    early elevations (up to 3 days) of S100B and ENO2 secondary to severe traumatic brain injury, predict deterioration to brain death, but more prominently associated with ENO2 than S100B
    Susceptibility to bipolar affective disorder with psychosis (BPAD)
    Variant & Polymorphism other
  • variants predisposing to a psychotic subtype of BPAD, possibly via alteration of gene expression
  • rs3788266, a functional promoter variant in the S100B gene where the presence of the G allele promotes increased gene expression and is associated with increased serum levels of the protein, strogly associated with BPAD
  • Candidate gene
  • for dyslexia
  • parameter of glial activation and/or death in several conditions of brain injury
  • Marker
  • S100A1 and S100B expression are marker to develop potency assays for cartilage regeneration cell therapies
  • Therapy target
  • mice deficient of both the S100a1 and S100b genes displayed normal skeletal growth from embryonic stage to adulthood