The 6-membered cyclic form of sugars is usually called the “pyranose” form in reference to the cyclic ether pyran.

TLS Online TPP Program

#Id: 7964

#XL - Q Biochemistry

Emphysema caused by a point mutation in -antitrypsin, which is normally secreted by hepatocytes and macrophages.

TLS Online TPP Program

#Id: 7963

#XL - Q Biochemistry

A hereditary form of emphysema illustrates the detrimental effects that can result from misfolding of proteins in the ER.

TLS Online TPP Program

#Id: 7962

#XL - Q Biochemistry

Hac1 activates transcription of genes encoding several protein-folding catalysts.

TLS Online TPP Program

#Id: 7961

#XL - Q Biochemistry

Quantity of free BiP in the ER lumen determines the relative proportions of monomeric and dimeric Ire1.

TLS Online TPP Program

#Id: 7959

#XL - Q Biochemistry

Unfolded-protein response

Key protein –Ire1 promotes formation of Hac1

TLS Online TPP Program

#Id: 7958

#XL - Q Biochemistry

The efficient formation of disulfide bonds in the lumen of the ER depends on the enzyme protein disulfide isomerase (PDI), which is present in all eukaryotic cells