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
|