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Lysosomal Hydrolases01:22

Lysosomal Hydrolases

Lysosomes are the site for the degradation of macromolecules and biological polymers released during membrane trafficking events such as secretory, endocytic, autophagic, and phagocytic pathways. The membrane-enclosed area of the lysosome, called the lumen, contains hydrolytic enzymes active in an acidic environment. These acid hydrolases are functional at a pH between 4.5 and 5 and are involved in cellular processes such as cell signaling, energy metabolism, restoration of the plasma membrane,...

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Comprehensive Autopsy Program for Individuals with Multiple Sclerosis
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Mapping Sanfilippo Syndrome: A Multisystem Clinicopathological Autopsy.

Mioara-Florentina Trandafirescu1, Elena-Roxana Avădănei1, Nina Filip1

  • 1"Grigore T. Popa" University of Medicine and Pharmacy, 16 University Street, 700115 Iasi, Romania.

Diagnostics (Basel, Switzerland)
|May 27, 2026
PubMed
Summary
This summary is machine-generated.

Mucopolysaccharidosis type III (MPS III), or Sanfilippo syndrome, is a systemic storage disorder affecting multiple organs, not just the brain. Autopsy revealed widespread glycosaminoglycan accumulation, impacting cardiovascular and visceral systems, contributing to mortality.

Area of Science:

  • Biochemistry
  • Pathology
  • Genetics

Background:

  • Mucopolysaccharidosis type III (MPS III), or Sanfilippo syndrome, is a rare genetic disorder.
Keywords:
Sanfilippo syndromeautopsyfoam cellsglycosaminoglycanshistopathologylysosomal storage disordermucopolysaccharidosis type IIImultisystem involvement

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  • It is characterized by deficiencies in enzymes crucial for heparan sulfate degradation.
  • While neurodegeneration is prominent, systemic glycosaminoglycan (GAG) accumulation is not fully understood.