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Proteostasis Deficiencies

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Disorders caused by imbalances in the protein homeostasis network - synthesis, folding, and transport of proteins; post-translational modifications; and degradation or clearance of misfolded proteins.

Also Known As:

Protein Folding Diseases; Protein Folding Disorders; Protein Misfolding Diseases; Proteostasis Dysfunctions; Deficiencies, Proteostasis; Deficiency, Proteostasis; Disease, Protein Folding; Disease, Protein Misfolding; Diseases, Protein Folding; Diseases, Protein Misfolding; Disorder, Protein Folding; Disorder, Protein Misfolding; Disorders, Protein Folding; Disorders, Protein Misfolding; Dysfunction, Proteostasis; Dysfunctions, Proteostasis; Folding Disease, Protein; Folding Diseases, Protein; Folding Disorder, Protein; Folding Disorders, Protein; Misfolding Disease, Protein; Misfolding Diseases, Protein; Misfolding Disorder, Protein; Misfolding Disorders, Protein; Protein Folding Disease; Protein Folding Disorder; Protein Misfolding Disease; Protein Misfolding Disorder; Proteostasis Deficiency; Proteostasis Dysfunction; Protein Misfolding Disorders

Networked: 234 relevant articles (9 outcomes, 8 trials/studies)

Relationship Network

Disease Context: Research Results

Nutritional and Metabolic Diseases: 2
- Metabolic Diseases: 3200
  - Proteostasis Deficiencies: 234
    - Amyloidosis: 7515
    - TDP-43 Proteinopathies: 129

Related Diseases

1.	Neurodegenerative Diseases (Neurodegenerative Disease)
2.	Alzheimer Disease (Alzheimer's Disease)
3.	Neoplasms (Cancer)
4.	Macular Degeneration (Age-Related Maculopathy)
5.	Eye Diseases (Eye Disease)

Experts

1.	Soto, Claudio: 5 articles (07/2015 - 03/2006)
2.	Carver, John A: 5 articles (11/2014 - 03/2008)
3.	Ramirez-Alvarado, Marina: 5 articles (01/2013 - 01/2009)
4.	Musgrave, Ian F: 4 articles (11/2014 - 09/2009)
5.	Kelly, Jeffery W: 4 articles (06/2013 - 12/2005)
6.	Alberti, Simon: 3 articles (05/2015 - 07/2011)
7.	Gitler, Aaron D: 3 articles (07/2014 - 01/2008)
8.	Dobson, Christopher M: 3 articles (06/2014 - 06/2011)
9.	Ghiso, Jorge: 3 articles (04/2014 - 07/2005)
10.	Huang, Kun: 3 articles (01/2014 - 08/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Proteostasis Deficiencies:

1.	Proteins (Proteins, Gene)IBA 07/03/2014 - "These findings reveal a mammalian system that removes misfolded proteins through sequential SUMOylation and ubiquitination and define its role in protection against protein-misfolding diseases. " 01/01/2008 - "Recent in vitro and in vivo studies suggest that destabilized proteins with defective folding induce aggregation and toxicity in protein-misfolding diseases. " 11/01/2015 - "In fact, the aggregation-prone and potentially harmful proteins at the origins of protein misfolding diseases are expressed throughout life, whereas the diseases are late onset. " 08/01/2015 - "Protein aggregation is associated with a number of protein misfolding diseases and is a major concern for therapeutic proteins. " 05/19/2015 - "Many protein-misfolding diseases are caused by proteins carrying prion-like domains. "
2.	Molecular Chaperones (Chaperone, Molecular)IBA 12/01/2011 - "In this short review, we focus on the molecular interplay between the macroautophagy system and molecular chaperones and highlight the relevance of the pathway mediated by BAG3 to aging and age-associated protein-misfolding diseases." 01/01/2011 - "The molecular chaperone network plays an important defensive role against cellular protein misfolding and has been identified as protective in experimental models of protein misfolding diseases like PD. " 02/01/2004 - "Roles of molecular chaperones in protein misfolding diseases." 01/01/2001 - "It is also characteristic for self-assembly of protein molecules from their unfolded fragments as well as for interaction of molecular chaperones with their substrates and it can be the origin of 'protein misfolding' diseases. " 08/21/2012 - "These data further implicate Hsp70-like molecular chaperones in protein misfolding disorders such as prion disease."
3.	AntibodiesIBA 08/01/2010 - "The PrioV anti-PrP antibodies could prove to be a valuable tool for the neutralization/clearance of PrP(Sc) in intracellular compartments of affected neurons and could potentially have wider applicability for the treatment of so-called protein-misfolding diseases." 07/15/2014 - "Potential candidate camelid antibodies for the treatment of protein-misfolding diseases." 05/01/2009 - "These antibodies may benefit the development of approaches towards prevention and treatment of protein misfolding diseases." 09/01/2008 - "The potential of intracellular antibodies for therapeutic targeting of protein-misfolding diseases." 01/01/2014 - "Antibodies that preferentially and specifically target pathological oligomeric protein and peptide assemblies, as opposed to their monomeric and amyloid counterparts, provide therapeutic and diagnostic opportunities for protein misfolding diseases. "
4.	TrehaloseIBA 06/01/2014 - "The repair of cellular homeostasis in many protein-misfolding diseases by trehalose is credited to the disaccharide being an inducer of autophagy, a mechanism by which aggregates of misfolded proteins are cleared by the cell. " 12/01/2010 - "Inhibition of aggregate formation as therapeutic target in protein misfolding diseases: effect of tetracycline and trehalose."
5.	Biological Markers (Surrogate Marker)IBA 04/10/2010 - "Specific areas covered include: 1) the operating principle and examples of AFM integrated with electrical recording, fluorescence imaging and microfluidics, (2) examples of AFM nanoscale imaging that has provided new paradigms in pathogenesis, including protein misfolding diseases (e.g., Alzheimer's disease, cancer, diabetes) and diseases arising from environmental and life choices and thus identifies potential therapeutic targets, (3) high-throughput parallel sensors, comprising integrated cantilevered microarrays, TIRF, microfluidics and nanoelectronics, for potential rapid diagnosis of pathogens, allergens and biomarkers as well as for therapeutics design, (4) the definition target macromolecules and structures, using intermolecular interaction assays, (5) the definition of abnormal vs normal tissues and the assessment of therapeutic efficacy by monitoring biomechanics, and (6) the development and characterization of nanocarrier-based drug delivery (e.g., nanoliposomes and nanoparticles) systems that allow high efficiency in vivo or the topical administration of a small dosage of therapeutics."
6.	Carbamazepine (Tegretol)FDA LinkGeneric 07/19/2013 - "We show that unlike that reported for several other protein misfolding diseases, carbamazepine did not correct KATP channel trafficking defects by activating autophagy; rather, it directly improved the biogenesis efficiency of mutant channels along the secretory pathway. "
7.	AllergensIBA 04/10/2010 - "Specific areas covered include: 1) the operating principle and examples of AFM integrated with electrical recording, fluorescence imaging and microfluidics, (2) examples of AFM nanoscale imaging that has provided new paradigms in pathogenesis, including protein misfolding diseases (e.g., Alzheimer's disease, cancer, diabetes) and diseases arising from environmental and life choices and thus identifies potential therapeutic targets, (3) high-throughput parallel sensors, comprising integrated cantilevered microarrays, TIRF, microfluidics and nanoelectronics, for potential rapid diagnosis of pathogens, allergens and biomarkers as well as for therapeutics design, (4) the definition target macromolecules and structures, using intermolecular interaction assays, (5) the definition of abnormal vs normal tissues and the assessment of therapeutic efficacy by monitoring biomechanics, and (6) the development and characterization of nanocarrier-based drug delivery (e.g., nanoliposomes and nanoparticles) systems that allow high efficiency in vivo or the topical administration of a small dosage of therapeutics."
8.	Amyloid (Amyloid Fibrils)IBA 05/30/2000 - "While thermodynamics suggest that inhibiting amyloid assembly would be difficult, the present study illustrates that the protein misfolding diseases are kinetically vulnerable to intervention." 12/01/2015 - "Thus, a greater understanding of amyloid fibre biology could enhance prospects of developing therapeutic interventions against this devastating class of protein-misfolding disorders. " 04/07/2015 - "Brain amyloid depositions are the main hallmarks of Alzheimer's and other protein misfolding diseases. " 06/01/2014 - "The amyloid state and its association with protein misfolding diseases." 04/01/2014 - "The protein conformational changes associated with the pathogenesis of protein misfolding disorders produce β sheet rich oligomers that are partially resistant to proteolysis and have a high tendency to form amyloid-like aggregates. "
9.	EnzymesIBA 01/01/2008 - "Our study could help in developing the strategy for enhancing the production of soluble industrial enzymes and finding the therapeutic agents against protein misfolding diseases." 06/01/2005 - "Stability is an interesting property of enzymes because (1) it is of great industrial importance, (2) it is relatively easy to screen for, and (3) the molecular basis of stability relates closely to contemporary issues in protein science such as the protein folding problem and protein folding diseases. "
10.	PerforinIBA 01/01/2010 - "The assembly of subunits in protein oligomers is an important topic to study as a vast number of proteins exists as stable or transient oligomer and because it is a mechanism used by some protein oligomers for killing cells (e.g., perforin from the human immune system, pore-forming toxins from bacteria, phage, amoeba, protein misfolding diseases, etc.). "

Therapies and Procedures

1.	Therapeutics 05/01/2014 - "Pharmacological chaperones hold great promise as potential therapeutics to ameliorate a variety of protein misfolding diseases. " 01/01/2014 - "Creating a path from the heat shock response to therapeutics of protein-folding diseases: an interview with Rick Morimoto." 10/24/2013 - "Combined with their amenability to protein engineering techniques to improve their stability and solubility, these findings support the suggestion that nanobodies can potentially be developed as therapeutics to combat protein misfolding diseases." 06/01/2012 - "Future studies in this direction will inform therapeutics development for cystic fibrosis and other protein misfolding diseases." 01/01/2010 - "This model of disease spread, which has analogies to other protein misfolding disorders such as prion disease, also suggests it may be possible to design assays for therapeutics that can inhibit fibril propagation and hence, possibly, disease progression."
2.	Immunotherapy 11/19/2012 - "Immunotherapy to proteins tightly associated with misfolded proteins might open up a new treatment option for many protein misfolding diseases."
3.	Topical Administration 04/10/2010 - "Specific areas covered include: 1) the operating principle and examples of AFM integrated with electrical recording, fluorescence imaging and microfluidics, (2) examples of AFM nanoscale imaging that has provided new paradigms in pathogenesis, including protein misfolding diseases (e.g., Alzheimer's disease, cancer, diabetes) and diseases arising from environmental and life choices and thus identifies potential therapeutic targets, (3) high-throughput parallel sensors, comprising integrated cantilevered microarrays, TIRF, microfluidics and nanoelectronics, for potential rapid diagnosis of pathogens, allergens and biomarkers as well as for therapeutics design, (4) the definition target macromolecules and structures, using intermolecular interaction assays, (5) the definition of abnormal vs normal tissues and the assessment of therapeutic efficacy by monitoring biomechanics, and (6) the development and characterization of nanocarrier-based drug delivery (e.g., nanoliposomes and nanoparticles) systems that allow high efficiency in vivo or the topical administration of a small dosage of therapeutics."
4.	Chinese Traditional Medicine (Traditional Chinese Medicine) 01/01/2011 - "Celastrol, a natural substance isolated from plant extracts used in traditional Chinese medicine, has been extensively investigated as a possible drug for treatment of cancer, autoimmune diseases, and protein misfolding disorders. "
5.	Drug Therapy (Chemotherapy) 03/01/2010 - "Because a large number of human diseases are protein folding diseases (approximately 30 to 50%), many researchers now realize that more directed approaches to target and reverse the fundamental misfolding reactions preceding disease are highly feasible and offer the potential of developing more targeted drug therapies. "