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Symbol S100B contributors: mct - updated : 24-04-2016
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
    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
    constitutional     --over  
    in patients with structural lesions resulting from mild traumatic brain injuries
    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
  • increased serum GFAP, S100B, ENO2 are associated with acute CO poisoning, and these biomarkers can be useful in assessing the clinical status of patients with CO poisoning
  • Therapy target
  • mice deficient of both the S100a1 and S100b genes displayed normal skeletal growth from embryonic stage to adulthood