Navigation Links

Proteopathy (Proteo- [pref. protein]; -pathy [suff. disease]; proteopathies pl.; proteopathic adj.). Proteopathy is the abnormal accumulation and toxicity of proteins in certain disease states.[1] Also, selective hyperproteolytic diseases have been referred to this category, e.g. critical illness myopathies or tumor cachexia.[2] The proteopathies comprise at least 30 diseases that affect a variety of organs and tissues, including Alzheimer's disease, Parkinson's disease, type 2 diabetes, amyloidosis, and a wide range of other disorders (see Table).[3][4]

The proteopathies are also called protein conformational diseases,[5] because a change in the 3-dimensional folding (conformation) of a protein increases the tendency of the protein to misfold and polymerize into aggregates that are resistant to clearance, and can become pathogenic. Because of the common structure of the polypeptide backbone, all proteins have the potential to misfold under some conditions.[6]

Only certain proteins are linked to proteopathy, possibly due to instability or other structural features of the monomeric protein that increase the probability of misconformation, which in nearly all instances involves an increase in beta-sheet secondary structure. Potential risk factors for proteopathic diseases augment the tendency of vulnerable proteins to self-assemble. They include destabilizing changes in the primary amino acid sequence of the protein, post-translational modifications (such as hyperphosphorylation), changes in temperature or pH, an increase in production of a protein, or a decrease in its clearance. Advancing age frequently is a risk factor.

In some proteopathies, abnormal assembly can be templated on an exogenous protein, typically a misfolded form of the same protein. In this way, the disease state can be induced in a susceptible host by the introduction of diseased tissue extract from an afflicted donor. The best known form of such infectious (or transmissible) proteopathy is prion disease, which can be transmitted by exposure of a host organism to purified prion protein in a disease-causing conformation.[7][8] There is now evidence that other proteopathies are inducible by a similar mechanism, including AA amyloidosis, apolipoprotein AII amyloidosis, and amyloidosis.[9][10] In all of these instances, an aberrant form of the protein itself appears to be the pathogenic agent.

Proteopathy Major aggregating protein
Alzheimer's disease, Cerebral β-amyloid angiopathy Amyloid β peptide (Aβ) (senile plaques), Tau protein (fibers)
Prion diseases (multiple) Prion protein
Parkinson's disease and other synucleinopathies (multiple) α-Synuclein
Tauopathies (multiple) Microtubule-associated protein tau
Frontotemporal lobar degeneration (FTLD) (Ubi+, Tau-) TDP-43
Amyotrophic lateral sclerosis (ALS) Superoxide dismutase, TDP-43
Huntington's disease and other triplet repeat disorders (multiple) Proteins with tandem glutamine expansions
Familial British dementia ABri
Familial Danish dementia ADan
Familial encephalopathy with neuroserpin inclusion bodies (FENIB) Neuroserpin
Hereditary cerebral hemorrhage with amyloidosis (Icelandic) (HCHWA-I) Cystatin C
Familial amyloidotic neuropathy, Senile systemic amyloidosis Transthyretin
AL (light chain) amyloidosis (primary systemic amyloidosis) Monoclonal immunoglobulin light chains
AH (heavy chain) amyloidosis Immunoglobulin heavy chains
AA (secondary) amyloidosis Amyloid A protein
Type II diabetes Islet amyloid polypeptide (IAPP; amylin)
Aortic medial amyloidosis Medin (lactadherin)
ApoAI amyloidosis Apolipoprotein AI
ApoAII amyloidosis Apolipoprotein AII
ApoAIV amyloidosis Apolipoprotein AIV
Finnish hereditary amyloidosis Gelsolin
Lysozyme amyloidosis Lysozyme
Fibrinogen amyloidosis Fibrinogen
Dialysis amyloidosis β2-microglobulin
Inclusion body myopathy/myositis Amyloid β peptide (Aβ)
Cataracts Crystallins
Medullary thyroid carcinoma Calcitonin
Cardiac atrial amyloidosis Atrial natriuretic factor
Pituitary prolactinoma Prolactin
Hereditary lattice corneal dystrophy Keratoepithelin
Cutaneous lichen amyloidosis Keratins
Corneal lactoferrin amyloidosis Lactoferrin
Pulmonary alveolar proteinosis Surfactant protein C (SP-C)
Critical illness myopathy (CIM) Hyperproteolytic state of myosin ubiquitination


  1. ^ Walker LC and LeVine H (2000). "The cerebral proteopathies". Neurobiol Aging 21: 559-561. PMID 10924770.
  2. ^ Friedrich O (2006). "Critical illness myopathy: what is happening?". Curr Opin Clin Nutr Metab Care 9: 403-409. PMID 16778569.
  3. ^ Walker LC, LeVine III H (2000). "The cerebral proteopathies: Neurodegenerative disorders of protein conformation and assembly". Mol Neurobiol 21: 83-95. PMID 11327151.
  4. ^ Chiti F, Dobson CM (2006). "Protein misfolding, functional amyloid, and human disease". Ann Rev Biochem 75: 333-366. PMID 16756495.
  5. ^ Carrell RW, Lomas DA (1997). "Conformational disease". Lancet 350: 134-138. PMID 9228977.
  6. ^ Dobson CM (1999). "Protein misfolding, evolution and disease". TIBS 24: 329-332. PMID 10470028.
  7. ^ Prusiner SB (2001). "Shattuck lecture—Neurodegenerative diseases and prions". N Engl J Med 344: 1516-1526. PMID 11357156.
  8. ^ Zou WQ, Gambetti P (2005). "From microbes to prions: the final proof of the prion hypothesis". Cell 121: 155-157. PMID 15851020.
  9. ^ Walker LC, LeVine H, Mattson MP, Jucker M (2006). "Inducible proteopathies". TINS 29: 438-443. PMID 16806508.
  10. ^ Meyer-Luehmann M, et al. (2006). "Exogenous induction of cerebral β-amyloidogenesis is governed by agent and host". Science 313: 1781-1784. PMID 16990547.


(Date:9/30/2014)... of Maryland-led research team has been awarded a ... of Health (NIH) to develop new imaging technologies ... understanding of how large networks of neurons in ... knowledge will help researchers identify the precise interactions ... like decision-making and speaking, and alterations in these ...
(Date:9/30/2014)... 2014 EverStryke , the permanent match ... along with a free book and class all about ... Michaels, prompting an investigative review. , “Joe Marshall of ... survival and preparation niche, and this product is an excellent ... and survival products that Joe offers on his website to ...
(Date:9/30/2014)... 30, 2014 Elevate , the latest ... better in and out of the gym by providing natural ... the attention of Shane Michaels, prompting an investigative review. ... the reason most people simply just don’t have the ability ... focused at work, getting the motivation to finish a project, ...
(Date:9/30/2014)... CA (PRWEB) September 30, 2014 ... providers in Turkey has gone up more than 79 ... The medical tourism company also reported total revenue tripled ... 2012. , The company’s professional strategy has allowed them ... — ultimately designed for patient comfort and privacy — ...
(Date:9/30/2014)... 30, 2014 Hospice & Palliative ... “In Celebration” gala, honoring three outstanding individuals for ... striving to provide extraordinary and dignified comfort, care ... serious or life-limiting illness. , The event was ... NY and recognized William (Bill) J. McGuinness, Director ...
Breaking Medicine News(10 mins):Health News:UMD receives inaugural BRAIN Initiative Award 2Health News:EverStryke: Review Exposes Joe Marshall’s Permanent Match Survival Product 2Health News:Elevate: Review Exposes Blue Star Nutraceutical’s Latest Supplement for Ultimate Brain Function, Focus, and Energy 2Health Announces Record Demand for Plastic Surgery in Turkey 2Health Announces Record Demand for Plastic Surgery in Turkey 3Health Announces Record Demand for Plastic Surgery in Turkey 4Health News:Hospice & Palliative Care of Westchester Honors Outstanding Individuals at Annual ‘In Celebration’ Gala 2
... New York, NY, August 6, 2009 Comprehensive health reform ... 13 million uninsured young adults ages 19-29 gain coverage, ... who now have coverage would not lose it, according ... coverage to all Americans through expansions in Medicaid and ...
... on medical tourism, exotic destinations , SATURDAY, Aug. 15 ... the U.S. government has a guidebook that could save ... and Prevention has released a new edition of ... as "The Yellow Book" for its distinctive cover, which ...
... , NEW YORK, Aug. 14 Healthcare may be on ... the price you pay at the pharmacy is actually getting the medicine ... to this report from Forest Laboratories at: _ ... Registered journalists can access video, audio, text, graphics and photos for ...
... , , , , ... Board: SNKTY), a Life Sciences company engaged in the research, development ... today announced revenues for its fiscal second quarter ended June 30, ... 31, 2009 and $691,000 reported in the comparable prior year period ...
... impact on cognition, study finds , FRIDAY, Aug. 14 ... difference in mental decline after the age of 70, ... the Aug. 1 issue of the American Journal ... background have an impact on cognitive functioning among the ...
... , Additional 28 New ... WASHINGTON, Aug. 14 Today, Secretary of Veterans Affairs Eric K. ... assistance in 28 additional communities across the country where the Department ... , , "VA is committed to providing high-quality ...
Cached Medicine News:Health News:Health reform proposals could help 13 million uninsured young adults gain coverage 2Health News:Health reform proposals could help 13 million uninsured young adults gain coverage 3Health News:International Travel Health Guidebook Gets Updated 2Health News:Senetek PLC Reports Second Quarter 2009 Financial Results 2Health News:Senetek PLC Reports Second Quarter 2009 Financial Results 3Health News:Senetek PLC Reports Second Quarter 2009 Financial Results 4Health News:Senetek PLC Reports Second Quarter 2009 Financial Results 5Health News:Senetek PLC Reports Second Quarter 2009 Financial Results 6Health News:Rich, Poor See Similar Declines in Old Age 2Health News:Secretary Shinseki Announces Expansion of Counseling for Combat Veterans 2
Other medicine definitionOther Tags