Published in Science on August 26, 1988
Direct sequencing from low-melt agarose with Sequenase. Nucleic Acids Res (1989) 2.09
Function and regulation of expression of pulmonary surfactant-associated proteins. Biochem J (1991) 1.73
Saposin A: second cerebrosidase activator protein. Proc Natl Acad Sci U S A (1989) 1.35
Biosynthesis and degradation of mammalian glycosphingolipids. Philos Trans R Soc Lond B Biol Sci (2003) 1.29
Identification of prosaposin as a neurotrophic factor. Proc Natl Acad Sci U S A (1994) 1.25
Distribution of saposin proteins (sphingolipid activator proteins) in lysosomal storage and other diseases. Proc Natl Acad Sci U S A (1990) 1.22
Characterization of a mutation in a family with saposin B deficiency: a glycosylation site defect. Proc Natl Acad Sci U S A (1990) 1.10
The role of saposin C in Gaucher disease. Mol Genet Metab (2012) 1.02
Neurological deficits and glycosphingolipid accumulation in saposin B deficient mice. Hum Mol Genet (2008) 0.99
The lysosomal proenzyme receptor that binds procathepsin L to microsomal membranes at pH 5 is a 43-kDa integral membrane protein. Proc Natl Acad Sci U S A (1993) 0.97
Insertion in the mRNA of a metachromatic leukodystrophy patient with sphingolipid activator protein-1 deficiency. Proc Natl Acad Sci U S A (1990) 0.95
Analysis of a splice-site mutation in the sap-precursor gene of a patient with metachromatic leukodystrophy. Am J Hum Genet (1996) 0.94
Developmental and tissue-specific expression of prosaposin mRNA in murine tissues. Am J Pathol (1994) 0.94
The protective role of prosaposin and its receptors in the nervous system. Brain Res (2014) 0.94
Increased gene expression of Alzheimer disease beta-amyloid precursor protein in senescent cultured fibroblasts. Proc Natl Acad Sci U S A (1991) 0.93
Lysosomal storage disorders: molecular basis and laboratory testing. Hum Genomics (2011) 0.90
Evidence for two cDNA clones encoding human GM2-activator protein. Biochem J (1992) 0.89
J3-crystallin of the jellyfish lens: similarity to saposins. Proc Natl Acad Sci U S A (2001) 0.89
Structure of saposin A lipoprotein discs. Proc Natl Acad Sci U S A (2012) 0.88
Effect of saposins on acid sphingomyelinase. Biochem J (1993) 0.86
The immunological functions of saposins. Adv Immunol (2010) 0.85
Acid sphingomyelinase possesses a domain homologous to its activator proteins: saposins B and D. Protein Sci (1994) 0.82
Complete localization of disulfide bonds in GM2 activator protein. Protein Sci (1998) 0.82
A stretch of 17 amino acids in the prosaposin C terminus is critical for its binding to sortilin and targeting to lysosomes. J Histochem Cytochem (2009) 0.80
Comparative study on glucocerebrosidase in spleens from patients with Gaucher disease. Biochem J (1990) 0.80
Correction of sulfatide metabolism after transfer of prosaposin cDNA to cultured cells from a patient with SAP-1 deficiency. Am J Hum Genet (1992) 0.79
A method to determine the kinetics of multiple proteins in human infants with respiratory distress syndrome. Anal Bioanal Chem (2012) 0.79
My journey into the world of sphingolipids and sphingolipidoses. Proc Jpn Acad Ser B Phys Biol Sci (2012) 0.79
Tissue-specific effects of saposin A and saposin B on glycosphingolipid degradation in mutant mice. Hum Mol Genet (2013) 0.79
Cloning, expression and map assignment of chicken prosaposin. Biochem J (1998) 0.78
Prosaposin overexpression following kainic acid-induced neurotoxicity. PLoS One (2014) 0.77
Invertebrate models of lysosomal storage disease: what have we learned so far? Invert Neurosci (2011) 0.76
A prosaposin-derived Peptide alleviates kainic Acid-induced brain injury. PLoS One (2015) 0.76
Deciphering alternative splicing and nonsense-mediated decay modulate expression in primary lymphoid tissues of birds infected with avian pathogenic E. coli (APEC). BMC Genet (2017) 0.75
A saposin deficiency model in Drosophila: Lysosomal storage, progressive neurodegeneration and sensory physiological decline. Neurobiol Dis (2016) 0.75
The arylsulphatase A gene and molecular genetics of metachromatic leucodystrophy. J Med Genet (1994) 0.75
Temporal changes in prosaposin expression in the rat dentate gyrus after birth. PLoS One (2014) 0.75
Isolation and quantification of soluble Alzheimer's beta-peptide from biological fluids. Nature (1992) 7.02
Primary structure of bovine pituitary basic fibroblast growth factor (FGF) and comparison with the amino-terminal sequence of bovine brain acidic FGF. Proc Natl Acad Sci U S A (1985) 4.60
Addressing multiple problems in the family practice office visit. J Fam Pract (2001) 3.36
Growth hormone-releasing factor from a human pancreatic tumor that caused acromegaly. Science (1982) 3.10
Molecular basis of different forms of metachromatic leukodystrophy. N Engl J Med (1991) 2.69
Niemann-Pick disease group C: clinical variability and diagnosis based on defective cholesterol esterification. A collaborative study on 70 patients. Clin Genet (1988) 2.50
Isolation and partial molecular characterization of pituitary fibroblast growth factor. Proc Natl Acad Sci U S A (1984) 2.46
A defect in cholesterol esterification in Niemann-Pick disease (type C) patients. Proc Natl Acad Sci U S A (1985) 2.38
Pituitary FSH is released by a heterodimer of the beta-subunits from the two forms of inhibin. Nature (1986) 2.37
Rho- and rac-dependent assembly of focal adhesion complexes and actin filaments in permeabilized fibroblasts: an essential role for ezrin/radixin/moesin proteins. J Cell Biol (1997) 2.35
Complementary DNA sequences of ovarian follicular fluid inhibin show precursor structure and homology with transforming growth factor-beta. Nature (1986) 2.17
Saposin proteins: structure, function, and role in human lysosomal storage disorders. FASEB J (1991) 1.84
Presence of somatostatin-28-(1-12) in hypothalamus and pancreas. Proc Natl Acad Sci U S A (1982) 1.66
Adult and infantile Gaucher disease in one family: mutational studies and clinical update. J Pediatr (1994) 1.58
Cloning and sequence analysis of cDNA for the precursor of human growth hormone-releasing factor, somatocrinin. Proc Natl Acad Sci U S A (1983) 1.57
Niemann-Pick C1 disease: the I1061T substitution is a frequent mutant allele in patients of Western European descent and correlates with a classic juvenile phenotype. Am J Hum Genet (1999) 1.57
Steroid sulfatase deficiency. Pediatr Res (1977) 1.56
Mutations in the arylsulfatase A pseudodeficiency allele causing metachromatic leukodystrophy. Am J Hum Genet (1991) 1.55
Molecular cloning of the rat integrin alpha 1-subunit: a receptor for laminin and collagen. J Cell Biol (1990) 1.54
Isolation and partial characterization of a Mr 32,000 protein with inhibin activity from porcine follicular fluid. Proc Natl Acad Sci U S A (1985) 1.54
Synthesis of active firefly luciferase by in vitro translation of RNA obtained from adult lanterns. Biochem Biophys Res Commun (1984) 1.53
Hematopoietic stem-cell transplantation in globoid-cell leukodystrophy. N Engl J Med (1998) 1.47
Type C Niemann-Pick disease. Abnormal metabolism of low density lipoprotein in homozygous and heterozygous fibroblasts. J Biol Chem (1986) 1.41
Macular cherry-red spots and myoclonus with dementia: coexistent neuraminidase and beta-galactosidase deficiencies. Biochem Biophys Res Commun (1978) 1.41
Molecular characterization of fibroblast growth factor: distribution and biological activities in various tissues. Recent Prog Horm Res (1986) 1.35
Saposin A: second cerebrosidase activator protein. Proc Natl Acad Sci U S A (1989) 1.35
Niemann-Pick C1 disease: correlations between NPC1 mutations, levels of NPC1 protein, and phenotypes emphasize the functional significance of the putative sterol-sensing domain and of the cysteine-rich luminal loop. Am J Hum Genet (2001) 1.34
Solid phase synthesis of somatostatin-28. Biochem Biophys Res Commun (1980) 1.34
Primary structure of the human follistatin precursor and its genomic organization. Proc Natl Acad Sci U S A (1988) 1.33
Primary structure of bovine brain acidic fibroblast growth factor (FGF). Biochem Biophys Res Commun (1985) 1.31
Isolation and partial characterization of follistatin: a single-chain Mr 35,000 monomeric protein that inhibits the release of follicle-stimulating hormone. Proc Natl Acad Sci U S A (1987) 1.29
A correlation of intracellular cerebroside sulfatase activity in fibroblasts with latency in metachromatic leukodystrophy. Biochem Biophys Res Commun (1971) 1.29
Cerebroside sulfatase activator deficiency induced metachromatic leukodystrophy. Am J Hum Genet (1981) 1.29
Prenatal diagnosis of metachromatic leukodystrophy in a family with pseudo arylsulfatase A deficiency by the cerebroside sulfate loading test. Pediatr Res (1980) 1.29
Retina- and eye-derived endothelial cell growth factors: partial molecular characterization and identity with acidic and basic fibroblast growth factors. Biochemistry (1985) 1.28
Human brain fibroblast growth factor. Isolation and partial chemical characterization. FEBS Lett (1985) 1.27
Type C Niemann-Pick disease. A parallel loss of regulatory responses in both the uptake and esterification of low density lipoprotein-derived cholesterol in cultured fibroblasts. J Biol Chem (1986) 1.27
Metachromatic leukodystrophy: arylsulfatase-A deficiency in skin fibroblast cultures. Proc Natl Acad Sci U S A (1969) 1.26
Nine cases of sphingomyelin lipidosis, a new variant in Spanish-American Children. Juvenile variant of Niemann-Pick Disease with foamy and sea-blue histiocytes. Am J Dis Child (1977) 1.25
Corpus luteum angiogenic factor is related to fibroblast growth factor. Endocrinology (1985) 1.23
Selective effects of somatostatin-14, -25 and -28 on in vitro insulin and glucagon secretion. Nature (1981) 1.23
Uridine diphospho-N-acetylgalactosamine-4-sulfate sulfohydrolase activity of human arylsulfatase B and its deficiency in the Maroteaux-Lamy syndrome. Biochem Biophys Res Commun (1975) 1.21
Cytogenetic, biochemical, and molecular analyses of a 22q13 deletion. Am J Med Genet (1992) 1.21
Purification and properties of D-mannitol-1-phosphate dehydrogenase and D-glucitol-6-phosphate dehydrogenase from Escherichia coli. J Bacteriol (1984) 1.20
Treatment of late infantile metachromatic leukodystrophy by bone marrow transplantation. N Engl J Med (1990) 1.19
Growth hormone releasing factor, somatocrinin, releases pituitary growth hormone in vitro. Proc Natl Acad Sci U S A (1982) 1.19
Acidic fibroblast growth factor (FGF) from bovine brain: amino-terminal sequence and comparison with basic FGF. EMBO J (1985) 1.18
Primary structure of ovine hypothalamic somatostatin-28 and somatostatin-25. Proc Natl Acad Sci U S A (1980) 1.18
Feline sphingolipidosis resembling Niemann-Pick disease type C. Acta Neuropathol (1990) 1.18
A protein activator of galactosylceramide beta-galactosidase. Biochim Biophys Acta (1982) 1.18
Characterization of the large deletion in the GALC gene found in patients with Krabbe disease. Hum Mol Genet (1995) 1.17
Immunological evidence for deficiency in an activator protein for sulfatide sulfatase in a variant form of metachromatic leukodystrophy. Proc Natl Acad Sci U S A (1983) 1.17
A new prosomatostatin-derived peptide reveals a pattern for prohormone cleavage at monobasic sites. Science (1987) 1.17
Electrophoretic forms of human liver alpha-L-fucosidase and their relationship to fucosidosis (mucopolysaccharidosis F). Clin Chim Acta (1974) 1.16
Metachromatic leukodystrophy without arylsulfatase A deficiency. Pediatr Res (1979) 1.15
Immunohistochemical detection of growth hormone-releasing factor in brain. Nature (1983) 1.15
Niemann-Pick disease: a genetic model in Siamese cats. Science (1980) 1.15
Controlling on-surface polymerization by hierarchical and substrate-directed growth. Nat Chem (2012) 1.14
Purification and properties of arylsulfatase. A from human urine. J Biol Chem (1975) 1.13
A large deletion together with a point mutation in the GALC gene is a common mutant allele in patients with infantile Krabbe disease. Hum Mol Genet (1995) 1.12
Alpha 1 beta 1 integrin heterodimer functions as a dual laminin/collagen receptor in neural cells. Biochemistry (1990) 1.12
Cerebroside sulfatase determination in cultured human fibroblasts. Biochim Biophys Acta (1972) 1.12
Characterization of a mutation in a family with saposin B deficiency: a glycosylation site defect. Proc Natl Acad Sci U S A (1990) 1.10
Mutational analysis in a patient with a variant form of Gaucher disease caused by SAP-2 deficiency. Somat Cell Mol Genet (1993) 1.09
Correction of feline arylsulphatase B deficiency (mucopolysaccharidosis VI) by bone marrow transplantation. Nature (1985) 1.08
cDNA clones coding for the structural subunit of a chicken brain nicotinic acetylcholine receptor. Neuron (1988) 1.08
Cerebroside sulfate hydrolysis by fibroblasts from a parent with metachromatic leukodystrophy. J Pediatr (1978) 1.08
An improved method for the identification of patients and carriers of Krabbe's disease. Clin Chim Acta (1974) 1.08
Isolation of an amino terminal extended form of basic fibroblast growth factor. Biochem Biophys Res Commun (1986) 1.07
Sialic acid storage disease with sialuria: clinical and biochemical features in the severe infantile type. Pediatrics (1983) 1.07
Two different mutations are responsible for Krabbe disease in the Druze and Moslem Arab populations in Israel. Hum Genet (1996) 1.07
Cloning and expression of cDNA encoding human galactocerebrosidase, the enzyme deficient in globoid cell leukodystrophy. Hum Mol Genet (1993) 1.07
Localization of the Krabbe disease gene (GALC) on chromosome 14 by multipoint linkage analysis. Am J Hum Genet (1993) 1.07
Human beta-mannosidase deficiency. N Engl J Med (1986) 1.06
Isolation, primary structure, and synthesis of human hypothalamic somatocrinin: growth hormone-releasing factor. Proc Natl Acad Sci U S A (1984) 1.06
Molecular genetics of metachromatic leukodystrophy. Hum Mutat (1994) 1.05
Isolation and structure of corticostatin peptides from rabbit fetal and adult lung. Proc Natl Acad Sci U S A (1988) 1.05
Neuronal nicotinic acetylcholine receptor beta-subunit is coded for by the cDNA clone alpha 4. FEBS Lett (1987) 1.05
Arylsulfatase B deficiency in Maroteaux-Lamy syndrome cultured fibroblasts. Biochem Biophys Res Commun (1974) 1.04
Feline Niemann-Pick disease type C. Am J Pathol (1994) 1.04
Structure of a novel human granulocyte peptide with anti-ACTH activity. Biochem Biophys Res Commun (1988) 1.04
Synthetic substrate beta-glucosidase activity in leukocytes: a reproducible method for the identification of patients and carriers of Gaucher's disease. Clin Genet (1978) 1.03
Detection of a point mutation in sphingolipid activator protein-1 mRNA in patients with a variant form of metachromatic leukodystrophy. Biochem Biophys Res Commun (1990) 1.02
Isolation and partial characterization of an endothelial cell growth factor from the bovine kidney: homology with basic fibroblast growth factor. Regul Pept (1985) 1.02
Four novel mutations in mucopolysaccharidosis type VII including a unique base substitution in exon 10 of the beta-glucuronidase gene that creates a novel 5'-splice site. Hum Mol Genet (1995) 1.02
Studies on the substrate specificity of hexosaminidase A and B from liver. Arch Biochem Biophys (1972) 1.02
Uptake and metabolism of radioactively labeled sphingomyelin in cultured skin fibroblasts from controls and patients with Niemann-Pick disease and other lysosomal storage diseases. Biochim Biophys Acta (1983) 1.02
Somatocrinin (growth hormone releasing factor) in vitro bioactivity; Ca++ involvement, cAMP mediated action and additivity of effect with PGE2. Biochem Biophys Res Commun (1982) 1.01
Presence of arylsulfatase A (ARS A) in multiple sulfatase deficiency disorder fibroblasts. Am J Hum Genet (1978) 1.01
Somatocrinin, growth hormone releasing factor, stimulates secretion of growth hormone in anesthetized rats. Biochem Biophys Res Commun (1982) 1.01
Assignment of the gene for human sphingolipid activator protein-2 (SAP-2) to chromosome 10. Am J Hum Genet (1985) 1.01
Molecular cloning of the sphingolipid activator protein-1 (SAP-1), the sulfatide sulfatase activator. Biochem Biophys Res Commun (1986) 1.01
Genetic galactocerebrosidase deficiency (globoid cell leukodystrophy, Krabbe disease) in rhesus monkeys (Macaca mulatta). Lab Anim Sci (1998) 1.00
Letter: Beta-galactosidase deficiency in young adults. Lancet (1974) 1.00
Concentrations of an activator protein for sphingolipid hydrolysis in liver and brain samples from patients with lysosomal storage diseases. J Clin Invest (1983) 1.00
Simplified procedure for preparation of 35S-labeled brain sulfatide. Lipids (1974) 1.00
Biosynthesis of the sulfatide/GM1 activator protein (SAP-1) in control and mutant cultured skin fibroblasts. Biochim Biophys Acta (1986) 1.00