PRKCSH
PRKCSH (Protein Kinase C Substrate 80K-H) is a protein that in humans is encoded by the PRKCSH gene. This protein is also known as Glucosidase II Subunit Beta and is involved in the process of protein folding and quality control within the endoplasmic reticulum (ER).
Function[edit | edit source]
The PRKCSH protein is a component of the glucosidase II complex, which is involved in protein folding and quality control in the ER. The glucosidase II complex is responsible for removing two glucose residues from N-linked glycans on newly synthesized proteins to prepare them for subsequent folding and modification. The PRKCSH subunit of the complex does not have glucosidase activity, but it is necessary for the stability and function of the complex.
Clinical significance[edit | edit source]
Mutations in the PRKCSH gene are associated with autosomal dominant polycystic liver disease (PCLD). PCLD is a rare genetic disorder characterized by the growth of numerous cysts in the liver, which can lead to various complications such as abdominal pain, liver failure, and bleeding cysts.
See also[edit | edit source]
References[edit | edit source]
 | This protein-related article is a stub. You can help WikiMD by expanding it. |
 | This genetics article is a stub. You can help WikiMD by expanding it. |
 | This medical article is a stub. You can help WikiMD by expanding it. |
Search WikiMD
Ad.Tired of being Overweight? Try W8MD's physician weight loss program.
Semaglutide (Ozempic / Wegovy and Tirzepatide (Mounjaro / Zepbound) available.
Advertise on WikiMD
|
WikiMD's Wellness Encyclopedia |
| Let Food Be Thy Medicine Medicine Thy Food - Hippocrates |
Translate this page: - East Asian
中文,
日本,
한국어,
South Asian
हिन्दी,
தமிழ்,
తెలుగు,
Urdu,
ಕನ್ನಡ,
Southeast Asian
Indonesian,
Vietnamese,
Thai,
မြန်မာဘာသာ,
বাংলা
European
español,
Deutsch,
français,
Greek,
português do Brasil,
polski,
română,
русский,
Nederlands,
norsk,
svenska,
suomi,
Italian
Middle Eastern & African
عربى,
Turkish,
Persian,
Hebrew,
Afrikaans,
isiZulu,
Kiswahili,
Other
Bulgarian,
Hungarian,
Czech,
Swedish,
മലയാളം,
मराठी,
ਪੰਜਾਬੀ,
ગુજરાતી,
Portuguese,
Ukrainian
Medical Disclaimer: WikiMD is not a substitute for professional medical advice. The information on WikiMD is provided as an information resource only, may be incorrect, outdated or misleading, and is not to be used or relied on for any diagnostic or treatment purposes. Please consult your health care provider before making any healthcare decisions or for guidance about a specific medical condition. WikiMD expressly disclaims responsibility, and shall have no liability, for any damages, loss, injury, or liability whatsoever suffered as a result of your reliance on the information contained in this site. By visiting this site you agree to the foregoing terms and conditions, which may from time to time be changed or supplemented by WikiMD. If you do not agree to the foregoing terms and conditions, you should not enter or use this site. See full disclaimer.
Credits:Most images are courtesy of Wikimedia commons, and templates Wikipedia, licensed under CC BY SA or similar.
Contributors: Prab R. Tumpati, MD
