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Molecular basis of GM1 gangliosidosis and Morquio disease, type B. Structure-function studies of lysosomal beta-galactosidase and the non-lysosomal beta-galactosidase-like protein.
  • J. Callahan
  • Biology, Medicine
  • Biochimica et biophysica acta
  • 8 October 1999
GM1 gangliosidosis and Morquio B disease are distinct disorders both clinically and biochemically yet they arise from the same beta-galactosidase enzyme deficiency. On the other hand,Expand
  • 96
  • 7
A Proteomic Analysis of Lysosomal Integral Membrane Proteins Reveals the Diverse Composition of the Organelle*S
Lysosomes are endocytic subcellular compartments that contribute to the degradation and recycling of cellular material. Using highly purified rat liver tritosomes (Triton WR1339-filled lysosomes) andExpand
  • 142
  • 6
Decreased elastin deposition and high proliferation of fibroblasts from Costello syndrome are related to functional deficiency in the 67-kD elastin-binding protein.
Costello syndrome is characterized by mental retardation, loose skin, coarse face, skeletal deformations, cardiomyopathy, and predisposition to numerous malignancies. The genetic origin of CostelloExpand
  • 113
  • 6
The Arf-family protein, Arl8b, is involved in the spatial distribution of lysosomes.
Lysosomes are late-endocytic organelles which primarily contribute to degradation and recycling of cellular material. From a previous proteomics study of purified rat liver lysosomal membranes weExpand
  • 73
  • 6
The 67-kD elastin/laminin-binding protein is related to an enzymatically inactive, alternatively spliced form of beta-galactosidase.
We and others have previously shown that a 67-kD cell surface elastin/laminin-binding protein (EBP) is responsible for cell adhesion to elastin and laminin and for mediating the process of elastinExpand
  • 160
  • 5
Presenilin-1, Nicastrin, Amyloid Precursor Protein, and γ-Secretase Activity Are Co-localized in the Lysosomal Membrane*
Alzheimer's disease (AD) is caused by the cerebral deposition of β-amyloid (Aβ), a 38–43-amino acid peptide derived by proteolytic cleavage of the amyloid precursor protein (APP). Initial studiesExpand
  • 288
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Membrane‐associated estrogen receptor and caveolin‐1 are present in central nervous system myelin and oligodendrocyte plasma membranes
The estrogen receptor (ER) is a member of a superfamily of ligand‐regulated transcription factors that were thought to localize primarily to the nucleus; however, a membrane‐associated ER that canExpand
  • 81
  • 5
Identification of the gene encoding the enzyme deficient in mucopolysaccharidosis IIIC (Sanfilippo disease type C).
Mucopolysaccharidosis IIIC (MPS IIIC), or Sanfilippo C, represents the only MPS disorder in which the responsible gene has not been identified; however, the gene has been localized to theExpand
  • 64
  • 5
The 67-kDa Enzymatically Inactive Alternatively Spliced Variant of β-Galactosidase Is Identical to the Elastin/Laminin-binding Protein*
Our previous studies showed immunological and functional similarities, as well as partial sequence homology, between the enzymatically inactive alternatively spliced variant of human β-galactosidaseExpand
  • 168
  • 4
The role of the endosomal/lysosomal system in amyloid-beta production and the pathophysiology of Alzheimer's disease: reexamining the spatial paradox from a lysosomal perspective.
One of the hallmarks of Alzheimer's disease is the cerebral deposition of plaques composed of a 37-43 amino acid amyloid-beta (Abeta) peptide. Abeta is produced by the sequential proteolytic cleavageExpand
  • 117
  • 4
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