Tauopathy

Immunohistochemical detection of hyperphosphorylated tau proteins in nerve cells in tauopathy

The group of tauopathies encompasses a group of neurodegenerative diseases , the common characteristic of which is the accumulation of tau proteins in the brain. The name Tau was chosen after the corresponding Greek letter .

Microscopic examination of the brain tissue of sick people reveals these tau protein deposits in nerve and glial cells . They often appear as neurofibrillary tangles (Engl. Neurofibrillary tangles ) on. They can be made visible with special stains (e.g. silver plating because they are argyrophilic structures) or immunohistochemically with antibodies against tau proteins. The electron microscope shows that there are different types of neurofibrillary tangles, depending on the disease. There are different tau deposition products depending on the isoform. A frequent classification is based on the number of specific repetitions at the microtubule binding site. Depending on the number of these tandem repeats, tauopathies are often further subdivided into 3R or 4R tauopathies.

3-dimensional structure of tau protein from the neurofibrillary tangles of an Alzheimer's patient, determined by cryo-electron microscopy (PDB code 5o3t). The short, relatively straight β-strands that are folded into a β-helix are typical of amyloids .

Tauopathies belong to the group of amyloidoses , in which proteins fold into an alternative structure, the β-helix . This can occur spontaneously (age-related) or as a result of inherited mutations in the protein (with significantly younger patients). The folded protein then precipitates as amyloid (so named by Rudolf Virchow because the particles, like starch with iodine tincture, give a blue color).

The cause of most tauopathies is unknown. Some tauopathies are hereditary. So one finds z. B. over 60 different mutations of the tau gene in patients with FTLD-MAPT.

The group of tauopathies includes:

• Dementia pugilistica , also known as chronic traumatic encephalopathy (CTE)
• FTLD-MAPT (formerly: FTDP-17 , Frontotemporal Dementia and Parkinsonism of Chromosome 17)
• Corticobasal Degeneration (CBD: 4R-Tau)
• Alzheimer's disease
• Pick's disease (3R tau)
• Neurofibrillary tangle dementia
• Progressive supranuclear palsy (PSP: 4R-Tau)
• Silver Grain Disease (AGD: 4R-Tau)
• Tauopathy with gliobular glial inclusions
• Unclassifiable tauopathies

See also

• Synucleinopathy
• Protein misfolding disease

literature

• Lebouvier T, Pasquier F, Buée L: Update on tauopathies . In: Curr Opin Neurol . tape 30 , no. 6 , December 2017, p. 589-598 , doi : 10.1097 / WCO.0000000000000502 , PMID 28914736 .

• Orr ME, Sullivan AC, Frost B: A Brief Overview of Tauopathy: Causes, Consequences, and Therapeutic Strategies. In: Trends Pharmacol Sci . tape 38 , no. 7 , July 2017, p. 637-648 , doi : 10.1016 / j.tips.2017.03.011 , PMID 28455089 .

• Wang Y, Mandelkow E: Tau in physiology and pathology. In: Nat Rev Neurosci . tape 17 , no. 1 , January 2016, p. 5–21 , doi : 10.1038 / nrn.2015.1 , PMID 26631930 .

Individual evidence

1. ↑ Rudolf Virchow: About a substance found in the human brain and spinal cord with the chemical reaction of cellulose . In: Archives for pathological anatomy and physiology and for clinical medicine . tape 6 , no. 1 , March 1, 1854, ISSN  0720-8723 , p. 135–138 , doi : 10.1007 / bf01930815 ( springer.com [accessed October 31, 2017]).

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !