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Disruption of a novel member of a sodium/hydrogen exchanger family and DOCK3 is associated with an attention deficit hyperactivity disorder-like phenotype. J Med Genet (2003) 1.62

The initial substrate-binding site of gamma-secretase is located on presenilin near the active site. Proc Natl Acad Sci U S A (2005) 1.59

Presenilin-1 affects trafficking and processing of betaAPP and is targeted in a complex with nicastrin to the plasma membrane. J Cell Biol (2002) 1.42

Positive and negative regulation of APP amyloidogenesis by sumoylation. Proc Natl Acad Sci U S A (2002) 1.20

Phosphorylation of amyloid precursor protein (APP) at Thr668 regulates the nuclear translocation of the APP intracellular domain and induces neurodegeneration. Mol Cell Biol (2006) 1.14

Presenilin-based genetic screens in Drosophila melanogaster identify novel notch pathway modifiers. Genetics (2006) 0.99

Transthyretin and the brain re-visited: is neuronal synthesis of transthyretin protective in Alzheimer's disease? Mol Neurodegener (2011) 0.99

The intramembrane cleavage site of the amyloid precursor protein depends on the length of its transmembrane domain. Proc Natl Acad Sci U S A (2002) 0.98

The presenilins turned inside out: implications for their structures and functions. Proc Natl Acad Sci U S A (2004) 0.97

A region within a lumenal loop of Saccharomyces cerevisiae Ycf1p directs proteolytic processing and substrate specificity. Eukaryot Cell (2003) 0.87

A novel mechanism for the regulation of amyloid precursor protein metabolism. J Cell Biol (2002) 0.86

Wnt5a controls Notch1 signaling through CaMKII-mediated degradation of the SMRT corepressor protein. J Biol Chem (2012) 0.85

Deducing the transmembrane domain organization of presenilin-1 in gamma-secretase by cysteine disulfide cross-linking. Biochemistry (2006) 0.84

Phosphorylation of nicastrin by SGK1 leads to its degradation through lysosomal and proteasomal pathways. PLoS One (2012) 0.80

Involvement of presenilin holoprotein upregulation in calcium dyshomeostasis of Alzheimer's disease. J Cell Mol Med (2013) 0.80

An early specific cell-cell interaction occurs in the production of beta-amyloid in cell cultures. Proc Natl Acad Sci U S A (2006) 0.78

An interactive network of elastase, secretases, and PAR-2 protein regulates CXCR1 receptor surface expression on neutrophils. J Biol Chem (2014) 0.78

Sortilin Fragments Deposit at Senile Plaques in Human Cerebrum. Front Neuroanat (2017) 0.75

Mutation of the beta-amyloid precursor protein in familial Alzheimer's disease increases beta-protein production. Nature (1992) 6.51

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Presenilin-dependent gamma-secretase processing of beta-amyloid precursor protein at a site corresponding to the S3 cleavage of Notch. EMBO Rep (2001) 2.70

Subcellular localization of wild-type and Parkinson's disease-associated mutant alpha -synuclein in human and transgenic mouse brain. J Neurosci (2000) 2.49

Enhanced production and oligomerization of the 42-residue amyloid beta-protein by Chinese hamster ovary cells stably expressing mutant presenilins. J Biol Chem (1997) 2.33

Constitutive phosphorylation of the Parkinson's disease associated alpha-synuclein. J Biol Chem (2000) 2.27

Intracellular generation and accumulation of amyloid beta-peptide terminating at amino acid 42. J Biol Chem (1997) 2.02

Presenilin proteins undergo heterogeneous endoproteolysis between Thr291 and Ala299 and occur as stable N- and C-terminal fragments in normal and Alzheimer brain tissue. Neurobiol Dis (1997) 1.92

Glycine 384 is required for presenilin-1 function and is conserved in bacterial polytopic aspartyl proteases. Nat Cell Biol (2000) 1.87

A loss of function mutation of presenilin-2 interferes with amyloid beta-peptide production and notch signaling. J Biol Chem (1999) 1.87

Selective insolubility of alpha-synuclein in human Lewy body diseases is recapitulated in a transgenic mouse model. Am J Pathol (2001) 1.81

Drosophila small cytoplasmic 19S ribonucleoprotein is homologous to the rat multicatalytic proteinase. Nature (1988) 1.80

Phosphorylation regulates intracellular trafficking of beta-secretase. J Biol Chem (2001) 1.74

The presenilin 2 mutation (N141I) linked to familial Alzheimer disease (Volga German families) increases the secretion of amyloid beta protein ending at the 42nd (or 43rd) residue. Proc Natl Acad Sci U S A (1997) 1.69

The role of APP processing and trafficking pathways in the formation of amyloid beta-protein. Ann N Y Acad Sci (1996) 1.67

The proteolytic fragments of the Alzheimer's disease-associated presenilin-1 form heterodimers and occur as a 100-150-kDa molecular mass complex. J Biol Chem (1998) 1.66

Activation of protein kinase C inhibits cellular production of the amyloid beta-protein. J Biol Chem (1993) 1.64

Intramembrane proteolysis by presenilins. Nat Rev Mol Cell Biol (2000) 1.58

Human presenilin-1, but not familial Alzheimer's disease (FAD) mutants, facilitate Caenorhabditis elegans Notch signalling independently of proteolytic processing. Genes Funct (1997) 1.42

Maturation and pro-peptide cleavage of beta-secretase. J Biol Chem (2000) 1.38

The Alzheimer's disease-associated presenilins are differentially phosphorylated proteins located predominantly within the endoplasmic reticulum. Mol Med (1996) 1.32

Deficiency of Aph1B/C-gamma-secretase disturbs Nrg1 cleavage and sensorimotor gating that can be reversed with antipsychotic treatment. Proc Natl Acad Sci U S A (2008) 1.26

The biological and pathological function of the presenilin-1 Deltaexon 9 mutation is independent of its defect to undergo proteolytic processing. J Biol Chem (1999) 1.21

Amyloidogenic function of the Alzheimer's disease-associated presenilin 1 in the absence of endoproteolysis. Biochemistry (1999) 1.16

Increased expression of beta-amyloid precursor protein during neuronal differentiation is not accompanied by secretory cleavage. Proc Natl Acad Sci U S A (1992) 1.15

The cell biology of Alzheimer's disease: uncovering the secrets of secretases. Curr Opin Neurobiol (2001) 1.15

Expression of Alzheimer's disease-associated presenilin-1 is controlled by proteolytic degradation and complex formation. J Biol Chem (1998) 1.13

Physiology and pathophysiology of alpha-synuclein. Cell culture and transgenic animal models based on a Parkinson's disease-associated protein. Ann N Y Acad Sci (2000) 1.12

Accumulation and aggregation of amyloid beta-protein in late endosomes of Niemann-pick type C cells. J Biol Chem (2000) 1.09

Presenilins are processed by caspase-type proteases. J Biol Chem (1997) 1.08

Zebrafish (Danio rerio) presenilin promotes aberrant amyloid beta-peptide production and requires a critical aspartate residue for its function in amyloidogenesis. Biochemistry (1999) 1.05

Presenilin-1 differentially facilitates endoproteolysis of the beta-amyloid precursor protein and Notch. Nat Cell Biol (2000) 1.04

The lysosomal cysteine protease, cathepsin S, is increased in Alzheimer's disease and Down syndrome brain. An immunocytochemical study. Am J Pathol (1995) 1.03

Degradation of amyloid beta-protein by a serine protease-alpha2-macroglobulin complex. J Biol Chem (1996) 1.00

Sensitivity to MPTP is not increased in Parkinson's disease-associated mutant alpha-synuclein transgenic mice. J Neurochem (2001) 0.98

Mutant presenilin 2 transgenic mouse: effect on an age-dependent increase of amyloid beta-protein 42 in the brain. J Neurochem (1998) 0.97

Protofibrils, the unifying toxic molecule of neurodegenerative disorders? Nat Neurosci (2001) 0.97

Cholesterol-dependent generation of a seeding amyloid beta-protein in cell culture. J Biol Chem (1999) 0.96

Separation of presenilin function in amyloid beta-peptide generation and endoproteolysis of Notch. Proc Natl Acad Sci U S A (2000) 0.96

Lysosomal processing of amyloid precursor protein to A beta peptides: a distinct role for cathepsin S. Biochem J (1995) 0.94

Phosphorylation of presenilin-2 regulates its cleavage by caspases and retards progression of apoptosis. Proc Natl Acad Sci U S A (1999) 0.94

Metabolism of the "Swedish" amyloid precursor protein variant in Madin-Darby canine kidney cells. J Biol Chem (1994) 0.94

Inhibition of amyloid beta-protein production in neural cells by the serine protease inhibitor AEBSF. Neuron (1996) 0.93

Proteolytic processing of the Alzheimer disease-associated presenilin-1 generates an in vivo substrate for protein kinase C. Proc Natl Acad Sci U S A (1997) 0.93

A loss of function mutant of the presenilin homologue SEL-12 undergoes aberrant endoproteolysis in Caenorhabditis elegans and increases abeta 42 generation in human cells. J Biol Chem (2000) 0.91

hFE65L influences amyloid precursor protein maturation and secretion. J Neurochem (1999) 0.91

Proteolytic fragments of the Alzheimer's disease associated presenilins-1 and -2 are phosphorylated in vivo by distinct cellular mechanisms. Biochemistry (1998) 0.91

A pathogenic presenilin-1 deletion causes abberrant Abeta 42 production in the absence of congophilic amyloid plaques. J Biol Chem (2000) 0.90

Specific increase in amyloid beta-protein 42 secretion ratio by calpain inhibition. Biochemistry (1997) 0.90

Caspase-mediated cleavage is not required for the activity of presenilins in amyloidogenesis and NOTCH signaling. Neuroreport (1998) 0.90

Elevated vulnerability to oxidative stress-induced cell death and activation of caspase-3 by the Swedish amyloid precursor protein mutation. J Neurosci Res (2001) 0.89

Fleecy amyloid deposits in the internal layers of the human entorhinal cortex are comprised of N-terminal truncated fragments of Abeta. J Neuropathol Exp Neurol (1999) 0.89

Brain expression of presenilins in sporadic and early-onset, familial Alzheimer's disease. Mol Med (2000) 0.88

Cholesterol-dependent generation of a unique amyloid beta-protein from apically missorted amyloid precursor protein in MDCK cells. Biochim Biophys Acta (1998) 0.88

Enhanced release of secreted form of Alzheimer's amyloid precursor protein from PC12 cells by nicotine. Mol Pharmacol (1997) 0.88

Cellular expression and proteolytic processing of presenilin proteins is developmentally regulated during neuronal differentiation. J Neurochem (1997) 0.87

Alpha-synuclein immunoreactive Lewy bodies and Lewy neurites in Parkinson's disease are detectable by an advanced silver-staining technique. Acta Neuropathol (1999) 0.86

Detection of distinct isoform patterns of the beta-amyloid precursor protein in human platelets and lymphocytes. Neurobiol Aging (1992) 0.86

Mutation of conserved aspartates affects maturation of both aspartate mutant and endogenous presenilin 1 and presenilin 2 complexes. J Biol Chem (2000) 0.86

Phosphorylation of the beta-amyloid precursor protein at the cell surface by ectocasein kinases 1 and 2. J Biol Chem (2000) 0.86

Genes and mechanisms involved in beta-amyloid generation and Alzheimer's disease. Eur Arch Psychiatry Clin Neurosci (1999) 0.84

Ectodomain phosphorylation of beta-amyloid precursor protein at two distinct cellular locations. J Biol Chem (1997) 0.84

Alzheimer's disease associated presenilin-1 holoprotein and its 18-20 kDa C-terminal fragment are death substrates for proteases of the caspase family. Biochemistry (1998) 0.84

The proteasome of Drosophila and features of the evolutionarily conserved PROS-gene family. Biomed Biochim Acta (1991) 0.83

Parkinson's pathology in a fly. Nature (2000) 0.82

UV light-induced autofluorescence of full-length Abeta-protein deposits in the human brain. Clin Neuropathol (2002) 0.82

Endoproteolysis of the ER stress transducer ATF6 in the presence of functionally inactive presenilins. Neurobiol Dis (2001) 0.82

Does failure of parkin-mediated ubiquitination cause juvenile parkinsonism? Trends Biochem Sci (2000) 0.82

Molecular characterization of the genomic regions of the Drosophila alpha-type subunit proteasome genes PROS-Dm28.1 and PROS-Dm35. Eur J Biochem (1992) 0.81

Apoptosis. Dead end for neurodegeneration? Nature (1999) 0.80

Alzheimer's disease. A technical KO of amyloid-beta peptide. Nature (1998) 0.80

Amyloid precursor protein in unique cholesterol-rich microdomains different from caveolae-like domains. Biochim Biophys Acta (2000) 0.80

Analysis of presenilin 1 and presenilin 2 expression and processing by newly developed monoclonal antibodies. J Neurosci Res (1999) 0.80

Lewy body variant of Alzheimer's disease: alpha-synuclein in dystrophic neurites of A beta plaques. Neuroreport (2000) 0.80

Posttranslational modifications of amyloid precursor protein : ectodomain phosphorylation and sulfation. Methods Mol Med (2000) 0.80

Proteolytic processing and degradation of Alzheimer's disease relevant proteins. Biochem Soc Trans (1999) 0.79

Nuclear signaling: a common function of presenilin substrates? J Mol Neurosci (2001) 0.79

Truncated presenilin 2 derived from differentially spliced mRNA does not affect the ratio of amyloid beta-peptide 1-42/1-40. Neuroreport (1998) 0.79

An in vivo assay for the identification of target proteases which cleave membrane-associated substrates. FEBS Lett (1999) 0.77

The emerging utility of animal models of chronic neurodegenerative diseases. Expert Opin Ther Targets (2001) 0.77

BACE1 Dependent Neuregulin Proteolysis. Curr Alzheimer Res (2011) 0.76

Mutation of conserved aspartates affect maturation of presenilin 1 and presenilin 2 complexes. Acta Neurol Scand Suppl (2000) 0.75

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