Crystallins, genes and cataract

  • Suraj P. Bhat
Part of the Progress in Drug Research book series (PDR, volume 60)


Far from being a physical entity, assembled of inanimate structural proteins, the ocular lens epitomizes the biological ingenuity that sustains an essential and near-perfect physical system of immaculate optics. Crystallins (α,β, and γ) provide transparency by dint of their high concentration, but it is debatable whether proteins that provide transparency are any different, biologically or structurally, from those that are present in non-transparent structures or tissues. It is becoming increasingly clear that crystallins may have a plethora of metabolic and regulatory functions, both within the lens as well as outside of it. α-Crystallins are members of a small heat shock family of proteins and β/γ-crystallins belong to the family of epidermis-specific differentiation proteins. Crystallin gene expression has been studied from the perspective of the lens specificity of their promoters. Mutations in α-, β-, and γ- crystallins are linked with the phenotype of the loss of transparency. Understanding catalytic, non-structural properties of crystallins may be critical for understanding the malfunction in molecular cascades that lead to cataractogenesis and its eventual therapeutic amelioration.

Key words

Crystallins gene expression promoters Pax6 stress promoter non-crystallin function small heat shock proteins AIM1 protein aggregation cataractogenesis age-related cataract congenital lens epithelium 

Glossary of abbreviations








αA CRY binding protein 1 (a transcription factor that binds to a promoter sequence in the αA gene)






high molecular mass β-crystallin


low molecular mass β-crystallin


base pair


cognate heat shock protein


γ-crystallins A–F




CCAAT enhancer binding protein


chloramphenicol acetyl transferase


Chinese hamster ovary


cellular musculoaponeurotic fibrosarcoma protein (a transcription factor)


monkey kidney cells (Cercopithecus aethiops, the African green monkey


cytoskeletal protein 49 kDa molecular mass


cyclic AMP Response Element


cyclic AMP response element binding protein


crystallin βB1,2,3


connexin 43


connexin 46


connexin 50


a sequence element in the αA crystallin promoter


Greek key motif


glycogen synthase kinase-3β


histone acetyl transferase


immortalized human lens epithelial cells-B3


heat shock element (a sequence in heat shock promoters)


heat shock factor (a transcription factor)

Hsp 16.5–90

heat shock protein 16.5–90 kDa molecular mass





LSR1, 2

lens specific region 1 and 2 found in the αB-crystallin gene promoter


Maf recognition element (see c-Maf above)

MIP 26

membrane intrinsic protein 26 kDa


nuclear factor kappa-B


nuclear factor-interleukin-6


paired box homeodomain-6 protein


paired box homeodomain-6 protein, a transcription factor


pituitary X3


protein retinoblastoma


homebox gene related to prospero gene of Drosophila


retinoic acid-responsive element


small heat shock protein

Sox1, 2, 3, Sineoculis 1, 2, 3

a transcription factor


Simian virus 40


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Copyright information

© Springer Basel AG 2003

Authors and Affiliations

  • Suraj P. Bhat
    • 1
  1. 1.Jules Stein Eye Institute and Brain Research InstituteGeffen School of Medicine at UCLALos AngelesUSA

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