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Human pathology in NCL.

Glenn W Anderson1, Hans H Goebel, Alessandro Simonati

  • 1Department of Histopathology, Great Ormond Street Hospital, London, WC1N 3JH, UK.

Biochimica Et Biophysica Acta
|December 4, 2012
PubMed
Summary
This summary is machine-generated.

Neuronal ceroid lipofuscinoses (NCL) are common childhood neurodegenerative diseases. Adult NCL forms, though less frequent, present distinct morphological features and genetic causes, requiring molecular analysis for diagnosis.

Keywords:
ADANCLANCLBrainCNSCTSDCVP(s)CVSCurvilinearEMEPEPMRERERGElectron microscopyExtracerebral tissuesFPP(s)FingerprintGROD(s)Granular osmiophilic depositsJNCLLFBLINCLLMMPS IIIANCLPASPMEPPT1RLP(s)SAP(s)SCMASTPP1adult-onset NCLautosomal-dominant adult NCLcentral nervous systemchorion villus samplecurvilinear profile(s)electroretinogramendoplasmic reticulumevoked potentialsfingerprint profile(s)gene encoding cathepsin Dgranular osmiophilic deposit(s)juvenile NCLlate-infantile NCLlight microscopyluxol fast bluemucopolysaccharidosis IIIAneuronal ceroid lipofuscinosispalmitoyl protein thioesterase 1periodic acid-Schiffprogressive epilepsy with mental retardationprogressive myoclonus epilepsyrectilinear profile(s)sphingolipid activator protein(s)subunit C of mitochondrial ATP synthasetransmission electron microscopytripeptidyl peptidase 1vLINCLvariant late-infantile NCL

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Area of Science:

  • Neuroscience
  • Genetics
  • Pathology

Background:

  • Neuronal ceroid lipofuscinoses (NCL) are the most frequent lysosomal and neurodegenerative diseases in childhood.
  • In adulthood, NCL represents a smaller fraction of neurodegenerative diseases, with distinct clinical and pathological characteristics.

Purpose of the Study:

  • To review the morphological and etiological aspects of NCL, focusing on adult-onset forms.
  • To highlight diagnostic markers and genetic underpinnings of various NCL subtypes.

Main Methods:

  • Morphological analysis of neuronal loss and lipopigment accumulation in cerebral and extracerebral tissues.
  • Review of genetic classifications and molecular links for different NCL forms, including adult-onset subtypes.

Main Results:

  • NCL is characterized by selective neuronal loss, primarily in cortical regions, and lipopigment accumulation in nerve and extracerebral cells.
  • Specific ultrastructural profiles (granular, curvilinear, fingerprint) aid in classifying NCL subtypes (e.g., CLN1, CLN2, CLN3).
  • Adult NCL includes autosomal-recessive forms linked to childhood genes (CLN1, CLN5, CLN6) and autosomal-dominant CLN4 (DNAJC5 gene).

Conclusions:

  • Accurate diagnosis of NCL subtypes relies on a combination of clinical presentation, morphological findings, and molecular genetic analysis.
  • Extracerebral tissues like lymphocytes and skin can provide diagnostic clues for NCL.
  • Understanding the genetic heterogeneity of adult NCL is crucial for accurate diagnosis and potential therapeutic strategies.