Page last updated: 2024-11-08

alanine and Glycogen Storage Disease Type II

alanine has been researched along with Glycogen Storage Disease Type II in 6 studies

Alanine: A non-essential amino acid that occurs in high levels in its free state in plasma. It is produced from pyruvate by transamination. It is involved in sugar and acid metabolism, increases IMMUNITY, and provides energy for muscle tissue, BRAIN, and the CENTRAL NERVOUS SYSTEM.
alanine : An alpha-amino acid that consists of propionic acid bearing an amino substituent at position 2.

Glycogen Storage Disease Type II: An autosomal recessively inherited glycogen storage disease caused by GLUCAN 1,4-ALPHA-GLUCOSIDASE deficiency. Large amounts of GLYCOGEN accumulate in the LYSOSOMES of skeletal muscle (MUSCLE, SKELETAL); HEART; LIVER; SPINAL CORD; and BRAIN. Three forms have been described: infantile, childhood, and adult. The infantile form is fatal in infancy and presents with hypotonia and a hypertrophic cardiomyopathy (CARDIOMYOPATHY, HYPERTROPHIC). The childhood form usually presents in the second year of life with proximal weakness and respiratory symptoms. The adult form consists of a slowly progressive proximal myopathy. (From Muscle Nerve 1995;3:S61-9; Menkes, Textbook of Child Neurology, 5th ed, pp73-4)

Research Excerpts

ExcerptRelevanceReference
"We report a male with late infantile glycogen storage disease type II (Pompe's disease) who presented at 12 months of age with muscular hypotonia and developmental delay."5.31L-alanine supplementation in late infantile glycogen storage disease type II. ( Bodamer, OA; Haas, D; Hermans, MM; Hoffmann, GF; Reuser, AJ, 2002)
"Late-onset acid maltase deficiency or glycogen storage disease type II (GSD II) is a rare disorder of intralysosomal glycogen metabolism, resulting in progressive myopathy that is secondary to increased muscle protein breakdown."2.40Dietary treatment in late-onset acid maltase deficiency. ( Bodamer, OA; Halliday, D; Leonard, JV, 1997)
"F."1.72L-alanine supplementation in Pompe disease (IOPD): a potential therapeutic implementation for patients on ERT? A case report. ( Banderali, G; Cefalo, G; Dionigi, AR; Ercoli, V; Paci, S; Piotto, M; Rovelli, V; Salvatici, E; Scopari, A; Zuvadelli, J, 2022)
"We report a male with late infantile glycogen storage disease type II (Pompe's disease) who presented at 12 months of age with muscular hypotonia and developmental delay."1.31L-alanine supplementation in late infantile glycogen storage disease type II. ( Bodamer, OA; Haas, D; Hermans, MM; Hoffmann, GF; Reuser, AJ, 2002)

Research

Studies (6)

TimeframeStudies, this research(%)All Research%
pre-19901 (16.67)18.7374
1990's1 (16.67)18.2507
2000's3 (50.00)29.6817
2010's0 (0.00)24.3611
2020's1 (16.67)2.80

Authors

AuthorsStudies
Rovelli, V1
Zuvadelli, J1
Piotto, M1
Scopari, A1
Dionigi, AR1
Ercoli, V1
Paci, S1
Cefalo, G1
Salvatici, E1
Banderali, G1
Bodamer, OA3
Haas, D1
Hermans, MM1
Reuser, AJ1
Hoffmann, GF1
Mundy, HR1
Williams, JE1
Cousins, AJ1
Lee, PJ1
Leonard, JV2
Halliday, D2
Umpleby, AM1
Trend, PS1
Chubb, D1
Conaglen, JV1
Williams, CD1
Hesp, R1
Scobie, IN1
Wiles, CM1
Spencer, G1
Sönksen, PH1

Clinical Trials (2)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Safety and Effectiveness of Resistance Exercise Training in Patients With Late Onset Pompe Disease- A Pilot Study[NCT02654886]10 participants (Actual)Interventional2015-10-31Completed
A Pilot Study of Diet and Exercise Therapy in Pompe Disease[NCT02363153]2 participants (Actual)Interventional2017-11-06Completed
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Reviews

1 review available for alanine and Glycogen Storage Disease Type II

ArticleYear
Dietary treatment in late-onset acid maltase deficiency.
    European journal of pediatrics, 1997, Volume: 156 Suppl 1

    Topics: Adolescent; Adult; Age of Onset; Alanine; Energy Metabolism; Glucose; Glycogen Storage Disease Type

1997
Dietary treatment in late-onset acid maltase deficiency.
    European journal of pediatrics, 1997, Volume: 156 Suppl 1

    Topics: Adolescent; Adult; Age of Onset; Alanine; Energy Metabolism; Glucose; Glycogen Storage Disease Type

1997
Dietary treatment in late-onset acid maltase deficiency.
    European journal of pediatrics, 1997, Volume: 156 Suppl 1

    Topics: Adolescent; Adult; Age of Onset; Alanine; Energy Metabolism; Glucose; Glycogen Storage Disease Type

1997
Dietary treatment in late-onset acid maltase deficiency.
    European journal of pediatrics, 1997, Volume: 156 Suppl 1

    Topics: Adolescent; Adult; Age of Onset; Alanine; Energy Metabolism; Glucose; Glycogen Storage Disease Type

1997

Trials

2 trials available for alanine and Glycogen Storage Disease Type II

ArticleYear
The effect of L-alanine therapy in a patient with adult onset glycogen storage disease type II.
    Journal of inherited metabolic disease, 2006, Volume: 29, Issue:1

    Topics: Aged; Alanine; Body Mass Index; Cross-Over Studies; Double-Blind Method; Female; Glycogen Storage Di

2006
The effect of a high protein diet on leucine and alanine turnover in acid maltase deficiency.
    Journal of neurology, neurosurgery, and psychiatry, 1989, Volume: 52, Issue:8

    Topics: Adult; Alanine; alpha-Glucosidases; Clinical Trials as Topic; Dietary Proteins; Female; Follow-Up St

1989

Other Studies

3 other studies available for alanine and Glycogen Storage Disease Type II

ArticleYear
L-alanine supplementation in Pompe disease (IOPD): a potential therapeutic implementation for patients on ERT? A case report.
    Italian journal of pediatrics, 2022, Mar-28, Volume: 48, Issue:1

    Topics: Alanine; alpha-Glucosidases; Dietary Supplements; Enzyme Replacement Therapy; Female; Glycogen Stora

2022
L-alanine supplementation in late infantile glycogen storage disease type II.
    Pediatric neurology, 2002, Volume: 27, Issue:2

    Topics: Administration, Oral; Alanine; Cardiomyopathies; Dietary Supplements; Glycogen Storage Disease Type

2002
The effects of l-alanine supplementation in late-onset glycogen storage disease type II.
    Neurology, 2000, Sep-12, Volume: 55, Issue:5

    Topics: Adolescent; Adult; Alanine; Energy Metabolism; Female; Glucose; Glycogen Storage Disease Type II; Hu

2000