PubRank

B I Brown

Author PubWeight™ 41.58‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Glycogen storage disease in adults. Ann Intern Med 1994 3.58
2 Portal diversion for the treatment of glycogen storage disease in humans. Ann Surg 1973 2.44
3 Liver transplantation for type IV glycogen storage disease. N Engl J Med 1991 2.31
4 Lack of an alpha-1,4-glucan: alpha-1,4-glucan 6-glycosyl transferase in a case of type IV glycogenosis. Proc Natl Acad Sci U S A 1966 1.72
5 The effect of partial portacaval transposition on the canine liver. Surgery 1967 1.54
6 Studies of lysosomal alpha-glucosidase. I. Purification and properties of the rat liver enzyme. Biochemistry 1970 1.49
7 The specific influence of nonhepatic splanchnic venous blood flow on the liver. Surg Forum 1965 1.34
8 The subcellular distribution of enzymes in type II glycogenosis and the occurrence of an oligo-alpha-1,4-glucan glucohydrolase in human tissues. Biochim Biophys Acta 1965 1.29
9 Immunoblot analyses of glycogen debranching enzyme in different subtypes of glycogen storage disease type III. J Pediatr 1990 1.22
10 Simultaneous absence of alpha-1,4-glucosidase and alpha-1,6-glucosidase activities (pH 4) in tissues of children with type II glycogen storage disease. Biochemistry 1970 1.22
11 Studies of lysosomal alpha-glucosidase. II. Kinetics of action of the rat liver enzyme. Biochemistry 1970 1.15
12 Hypoglycemia, hepatic dysfunction, muscle weakness, cardiomyopathy, free carnitine deficiency and long-chain acylcarnitine excess responsive to medium chain triglyceride diet. Pediatr Res 1983 1.13
13 Preparation and properties of the glycogen-debranching enzyme from rabbit liver. Biochim Biophys Acta 1972 1.13
14 A new variant of type IV glycogenosis: deficiency of branching enzyme activity without apparent progressive liver disease. Hepatology 1988 1.06
15 Juvenile polysaccharidosis with cardioskeletal myopathy. Arch Pathol Lab Med 1987 1.04
16 Studies of the residual glycogen branching enzyme activity present in human skin fibroblasts from patients with type IV glycogen storage disease. Biochem Biophys Res Commun 1983 1.00
17 Administration of a mixture of fungal glucosidases to a patient with type II glycogenosis (Pompe's disease). Pediatrics 1968 0.97
18 The molecular heterogeneity of purified human liver lysosomal alpha-glucosidase (acid alpha-glucosidase). Arch Biochem Biophys 1978 0.93
19 Hepatorenal glycogenosis (type I glycogenosis) and carcinoma of the liver. J Pediatr 1969 0.92
20 Portal diversion in glycogen storage disease. Surgery 1969 0.91
21 Clinical management of the phencyclidine psychosis. Clin Toxicol 1976 0.91
22 Effect of changes in the outer structure of glycogen on the debranching activity of the transferase-glucosidase system. Arch Biochem Biophys 1966 0.91
23 Pompe's disease: detection of heterozygotes by lymphocyte stimulation. Science 1969 0.91
24 Liver transplantation for type I and type IV glycogen storage disease. Eur J Pediatr 1993 0.89
25 A mild form of muscular glycogenosis in two brothers with alpha-1, 4-glucosidase deficiency. Ann Paediatr 1965 0.88
26 The experimental production of glycogen storage in cultured human fibroblasts. Biochem Biophys Res Commun 1972 0.87
27 Definitive prenatal diagnosis for type III glycogen storage disease. Am J Hum Genet 1990 0.85
28 Peripheral nerve in type III glycogenosis: selective involvement of unmyelinated fiber Schwann cells. Muscle Nerve 1985 0.85
29 Type IV glycogen storage disease: branching enzyme deficiency in skin fibroblasts and possible heterozygote detection. J Pediatr 1971 0.85
30 Glycogen debranching enzyme: purification, antibody characterization, and immunoblot analyses of type III glycogen storage disease. Am J Hum Genet 1987 0.83
31 The apparent activity in vivo of the lysosomal pathway of glycogen catabolism in cultured human skin fibroblasts from patients with type III glycogen storage disease. J Biol Chem 1978 0.82
32 Hepatic fructose-1,6-diphosphatase deficiency. A cause of lactic acidosis and hypoglycemia in infancy. J Clin Invest 1972 0.81
33 Clinical and laboratory observations in a child with hepatic phosphorylase kinase deficiency. Metabolism 1986 0.80
34 X-linked glycogen storage disease. A cause of hypotonia, hyperuricemia, and growth retardation. Am J Dis Child 1985 0.78
35 A comparison of applicants and applications to Canadian medical and dental schools, 1965-66. Can Med Assoc J 1966 0.75
36 Alpha-1,4-glucosidase activity in leucocytes from the family of two brothers who lack this enzyme in muscle. Biochem J 1966 0.75
37 Metabolic and biochemical studies in fructose 1,6-diphosphatase deficiency. J Pediatr 1973 0.75
38 Studies on the structure and mechanism of action of the glycogen debranching enzymes of muscle nd liver. Ann N Y Acad Sci 1973 0.75
39 Excretion of 14C in urine of the domestic sow after injection of radioactive estradiol-17 beta, esterone, corticosterone and cortisol. J Anim Sci 1970 0.75
40 Diphenylhydantoin treatment. Arch Intern Med 1975 0.75
41 Structure of the polysaccharide formed by incubating glycogen with D-[14C]glucose in the presence of the glycogen debranching enzyme [amylo-(1 linked to 6)-glucosidase-4-alpha-glucanotransferase]. Carbohydr Res 1978 0.75
42 Glycogen storage disease type IB. Arch Pathol Lab Med 1983 0.75
43 Realistic workloads for community health nurses. Nurs Outlook 1980 0.75
44 Lipid storage myopathy in infantile Pompe's disease. Arch Neurol 1982 0.75
45 Neutropenia in a patient with type IB glycogen storage disease: in vitro response to lithium chloride. J Pediatr 1980 0.75
46 The Canadian Dental Association dental health plan for children. Can J Public Health 1969 0.75