Evidence map›Paper›PMID 27656840›Full record

Trial reportAutophagy2016

Autophagy activation in COL6 myopathic patients by a low-protein-diet pilot trial.

Silvia Castagnaro, Camilla Pellegrini, Massimo Pellegrini, Martina Chrisam, Patrizia Sabatelli, Silvia Toni, Paolo Grumati, Claudio Ripamonti, Loredana Pratelli, Nadir M Maraldi and 6 more

Registry-linked trialOpen access · hybridAbstract readClinical Trial
In one paragraph

Trial report in Autophagy, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT01438788 (Low Protein Diet to Correct Defective Autophagy in Patients With Collagen VI Related Myopathies), which is not on this map. Cited by 30 papers.

0numbers the graph read from it
0cells of the map it votes in
30citing papers in PubMed
4.3field-weighted citation impact, top 5% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

NCT01438788 phase2completednot on this map

Low Protein Diet to Correct Defective Autophagy in Patients With Collagen VI Related Myopathies

TypeinterventionalSponsorIstituto Ortopedico RizzoliRan2011 to 2013Enrolled8ConditionsBethlem Myopathy, Ullrich Congenital Muscular DystrophyArmsLow protein diet
3 · Its place in the literature

Who cites it

30 citing papers in PubMed, 55 citations in OpenAlex.

  1. Review
  2. Review
  3. Review
  4. Article
  5. Article
  6. Article
  7. Article
  8. Article
  9. Collagen VI in the Musculoskeletal System.International journal of molecular sciences · 2023
    Review
  10. Article
  11. Autophagy in striated muscle diseases.Frontiers in cardiovascular medicine · 2022
    Review
  12. Article
  13. Article
  14. Article
  15. Article
  16. Review
  17. Review
  18. Article
  19. Skeletal muscle in health and disease.Disease models & mechanisms · 2020
    Review
  20. Autophagy in the Regulation of Tissue Differentiation and Homeostasis.Frontiers in cell and developmental biology · 2020
    Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

16 authors at 5 institutions in 2 countries.

Silvia Castagnaroa Department of Molecular Medicine , University of Padova , Padova , Italy.
Camilla Pellegrinib Laboratory of Musculoskeletal Cell Biology, Rizzoli Orthopedic Institute , Bologna , Italy.
Massimo Pellegrinic Department of Diagnostic , Clinical and Public Health Medicine, University of Modena and Reggio Emilia , Modena , Italy.
Martina Chrisama Department of Molecular Medicine , University of Padova , Padova , Italy.
Patrizia Sabatellib Laboratory of Musculoskeletal Cell Biology, Rizzoli Orthopedic Institute , Bologna , Italy.
Silvia Tonic Department of Diagnostic , Clinical and Public Health Medicine, University of Modena and Reggio Emilia , Modena , Italy.
Paolo Grumatie Institute of Biochemistry II, Goethe University School of Medicine , Frankfurt am Main , Germany.
Claudio Ripamontif Department of Medicine and Rheumatology , University of Bologna , Bologna , Italy.
Loredana Pratellig Clinical Pathology Unit, Rizzoli Orthopedic Institute , Bologna , Italy.
Nadir M Maraldib Laboratory of Musculoskeletal Cell Biology, Rizzoli Orthopedic Institute , Bologna , Italy.
Daniela Cocchih Department of Statistical Sciences , University of Bologna , Bologna , Italy.
Valeria Righii Department of Life Quality Studies , Campus Rimini, University of Bologna , Bologna , Italy.
Cesare Faldinij Department of Orthopedics , University of Bologna , Bologna , Italy.
Marco Sandrik Department of Biomedical Sciences , University of Padova , Padova , Italy.
Paolo Bonaldoa Department of Molecular Medicine , University of Padova , Padova , Italy.
Luciano Merlinib Laboratory of Musculoskeletal Cell Biology, Rizzoli Orthopedic Institute , Bologna , Italy.
Istituto Ortopedico Rizzoli · ITUniversity of Bologna · ITUniversity of Padua · ITUniversity of Modena and Reggio Emilia · ITGoethe University Frankfurt · DE

Funding

Telethon GGP14202
6 · The paper itself

Abstract

A pilot clinical trial based on nutritional modulation was designed to assess the efficacy of a one-year low-protein diet in activating autophagy in skeletal muscle of patients affected by COL6/collagen VI-related myopathies. Ullrich congenital muscular dystrophy and Bethlem myopathy are rare inherited muscle disorders caused by mutations of COL6 genes and for which no cure is yet available. Studies in col6 null mice revealed that myofiber degeneration involves autophagy defects and that forced activation of autophagy results in the amelioration of muscle pathology. Seven adult patients affected by COL6 myopathies underwent a controlled low-protein diet for 12 mo and we evaluated the presence of autophagosomes and the mRNA and protein levels for BECN1/Beclin 1 and MAP1LC3B/LC3B in muscle biopsies and blood leukocytes. Safety measures were assessed, including muscle strength, motor and respiratory function, and metabolic parameters. After one y of low-protein diet, autophagic markers were increased in skeletal muscle and blood leukocytes of patients. The treatment was safe as shown by preservation of lean:fat percentage of body composition, muscle strength and function. Moreover, the decreased incidence of myofiber apoptosis indicated benefits in muscle homeostasis, and the metabolic changes pointed at improved mitochondrial function. These data provide evidence that a low-protein diet is able to activate autophagy and is safe and tolerable in patients with COL6 myopathies, pointing at autophagy activation as a potential target for therapeutic applications. In addition, our findings indicate that blood leukocytes are a promising noninvasive tool for monitoring autophagy activation in patients.

Indexed as

AutophagyDiet, Protein-RestrictedAdultAlanineBiomarkersBiopsyBody CompositionCollagen Type VIContractureFemaleHumansLactic AcidLeukocytesMaleMiddle AgedMitochondriaAlanineBiomarkersCollagen Type VILactic AcidautophagyBethlem myopathyclinical trialcollagen VIlow-protein dietmuscular dystrophiesUllrich congenital muscular dystrophy

Identifiers

PMID27656840
PMCPMC5173266
OpenAlexW2522369681

What OpenQuestion holds

Textmetadata
LicenceCC BY
Read underepoch 390

Registered trials

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.