Alpha-1-antitrypsin in duodenal fluid and gallbladder bile

Kyaw-Myint, T. O.; Howell, A.; Murphy, Gérard M.; Anderson, Charlotte M.

doi:10.1016/0009-8981(75)90217-x

Cited by 15 publications

(7 citation statements)

References 10 publications

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“…Alternatively, conceivable alterations in bile compositions by the Z allele might, on a speculative basis, promote inflammation‐driven biliary carcinogenesis. Indeed, α1AT is present in bile at low levels, however, it appears to be mostly nonfunctional 20, 21 . Taken together, the potential role of α1AT in biliary physiology and pathophysiology remains to be defined.…”

Section: Discussionmentioning

confidence: 99%

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Heterozygosity for the alpha1-antitrypsin Z allele may confer genetic risk of cholangiocarcinoma

Mihalache

Höblinger

Grünhage

et al. 2010

Alimentary Pharmacology &Amp; Therapeutics

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Section: Discussionmentioning

confidence: 99%

“…Indeed, a1AT is present in bile at low levels, however, it appears to be mostly nonfunctional. 20,21 Taken together, the potential role of a1AT in biliary physiology and pathophysiology remains to be defined.…”

Section: Discussionmentioning

confidence: 99%

Heterozygosity for the alpha1-antitrypsin Z allele may confer genetic risk of cholangiocarcinoma

Mihalache

Höblinger

Grünhage

et al. 2010

Alimentary Pharmacology &Amp; Therapeutics

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“…(ii) Biliary cells (Hering and bile duct cells) synthesize and export AAT into the biliary system and contribute to the bile AAT pool that is estimated to represent 30% of the circulating AAT [29]. The capability of biliary cells for AAT synthesis has been discovered from studies of AATD deficient livers in which biliary cells have shown immunohistochemical and EM evidence of simultaneous accumulation of Z AAT in hepatocytes and biliary cells from the same liver [30] (Figure 3).…”

Section: Hepatic Source and Function Of Aatmentioning

confidence: 99%

“…These data, together with the clinical observation that AATD (Pi ZZ) cirrhotic patients, after liver transplantation from a Pi MM donor, acquire the phenotype of the donor and normalize the serum levels [27], have definitely established that the liver is the only source of the circulating AAT [28]. (ii) Biliary cells (Hering and bile duct cells) synthesize and export AAT into the biliary system and contribute to the bile AAT pool that is estimated to represent 30% of the circulating AAT [29]. The capability of biliary cells for AAT synthesis has been discovered from studies of AATD deficient livers in which biliary cells have shown immunohistochemical and EM evidence of simultaneous accumulation of Z AAT in hepatocytes and biliary cells from the same liver [30] (Figure 3).…”

Section: Hepatic Source and Function Of Aatmentioning

confidence: 99%

Hepatic and Extrahepatic Sources and Manifestations in Endoplasmic Reticulum Storage Diseases

Callea

Francalanci

Giovannoni

2021

IJMS

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Alpha-1-antitrypsin (AAT) and fibrinogen are secretory acute phase reactant proteins. Circulating AAT and fibrinogen are synthesized exclusively in the liver. Mutations in the encoding genes result in conformational abnormalities of the two molecules that aggregate within the rough endoplasmic reticulum (RER) instead of being regularly exported. That results in AAT-deficiency (AATD) and in hereditary hypofibrinogenemia with hepatic storage (HHHS). The association of plasma deficiency and liver storage identifies a new group of pathologies: endoplasmic reticulum storage disease (ERSD).

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“…This would suggest a secretion into bile rather than towards the blood. Since AAT represents a normal component of human bile (Kyaw-Myint et al 1975), dynamic investigations should clarify whether bile duct cells contribute to biliary AAT. Finally, the findings from this study might explain the presence of AAT in cholangiocarcinoma as shown by others (Palmer & Wolfe 1976, Reintoft & Hagerstrand 1979, reflecting its presence in normal ductular and ductal cells.…”

Section: Discussionmentioning

confidence: 99%

Storage of alpha‐1‐antitrypsin in intrahepatic bile duct cells in alpha‐1‐antitrypsin deficiency (Pi Z phenotype)

et al. 1985

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Storage of alpha-l-antitrypsin in intrahepatic bile duct cells in alpha-l-antitrypsin deficiency (Pi Z phenotype)Storage of alpha-l-antitrypsin (AAT) has been found in a small number of bile duct cells in liver tissue specimens from patients with Pi MZ, Pi SZ and Pi ZZ phenotypes. The storage appeared in the form of intracellular AAT immunoreactive inclusions. On EM investigation, AAT-like material was detected within cisternae of the RER and SER. Such AAT inclusions were found in proliferating bile ductules in conditions such as cirrhosis, focal nodular hyperplasia and extrahepatic obstruction. They were also observed in normal biliary structures at the level of the canals of Hering, bile ductules and interlobular ducts in 13 out of 47 cases. These findings are interpreted as indicating that the intrahepatic bile duct cells are a further source of AAT, and that in case of defective export of AAT from the cell, as is the case for the Z protein, the protein accumulates not only in hepatocytes but in biliary cells as well.

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Alpha-1-antitrypsin in duodenal fluid and gallbladder bile

Cited by 15 publications

References 10 publications

Heterozygosity for the alpha1-antitrypsin Z allele may confer genetic risk of cholangiocarcinoma

Heterozygosity for the alpha1-antitrypsin Z allele may confer genetic risk of cholangiocarcinoma

Hepatic and Extrahepatic Sources and Manifestations in Endoplasmic Reticulum Storage Diseases

Storage of alpha‐1‐antitrypsin in intrahepatic bile duct cells in alpha‐1‐antitrypsin deficiency (Pi Z phenotype)

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