Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.1.3.1 (alkaline phosphatase)
 47,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The presence of alpha-1-fetoprotein, the heat stable alkaline phosphatase and Australia antigen was examined in 103 patients with porphyria cutanea tarda, 300 patients with cirrhosis and 18 patients with primary liver carcinoma. The heat stable alkaline phosphatase was determined in 46 percent of patients with porphyria cutanea tarda and in 61 percent of patients with primary liver carcinoma. Alpha-1-fetoprotein was detected in 61 percent of patients with primary liver carcinoma and in 2 patients with porphyria cutanea tarda in whom primary liver carcinoma was proved later. The simultaneous occurrence of alpha-1-fetoprotein and the heat stable alkaline phosphatase was found in 50 percent of cases with primary liver carcinoma. Neither the patients with porphyria cutanea tarda nor the patients with cirrhosis were Australia-antigen positive. Australia-antigen could be detected only in one patient with alpha-1-fetoprotein positive-carcinoma of the liver.
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PMID:Alpha-1-fetoprotein and the heat stable alkaline phosphatase in some liver diseases. 4 25

A series of teratocarcinoma-thymus hybrid cells (PCT hybrids), which had been shown previously to give rise to multidifferentiated tumors and hence to be pluripotent, was tested to see whether these cells resembled their embryonal carcinoma parent in other ways as well. PCT hybrid cells looked like embryonal carcinoma parent in other ways as well. PCT hybrid cells looked like embryonal carcinoma cells by phase contrast and electron microscopy, have high levels of alkaline phosphatase, and fail to express Thy 1 alloantigen (which is present on thymocyte parental cells, but not on embryonal carcinoma cells). PCT hybrids do, however, exhibit H2 antigens, which are present only at very low levels, if at all, on embryonal carcinoma cells.
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PMID:Properties of teratocarcinoma-thymus somatic cell hybrids. 60 85

Vitronectin, identical with serum-spreading factor and S-protein of complement, is a glycoprotein present in both plasma and tissue. It stimulates cell adhesion and spreading and affects the complement and coagulation pathways. Vitronectin immunoreactivity was recently found in conjunction with dermal and renal elastic fibres, in renal amyloid deposits in cases of AL- and AA-amyloidosis, and in sclerotic glomerular lesions. Skin specimens from lesions of patients with selected skin diseases were investigated with an avidin-biotin peroxidase technique using both monoclonal and polyclonal anti-vitronectin antibodies and an alkaline phosphatase anti-alkaline phosphatase technique using monoclonal anti-vitronectin antibodies. Vitronectin immunoreactivity was found in association with the abnormal elastic tissue in solar elastosis and pseudoxanthoma elasticum. It was also found in conjunction with dermal amyloid deposits in primary localized cutaneous amyloidosis and in Civatte bodies in cases of lichen ruber planus. In cases of erythropoietic protoporphyria and porphyria cutanea tarda, hyaline perivascular deposits also demonstrated positive vitronectin immunoreactivity. The presence of vitronectin immunoreactivity not only in normal and degenerated elastic fibres but also in various pathological tissue deposits suggests that vitronectin occurs both in elastic fibres and in different types of abnormal protein deposits.
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PMID:Immunohistochemical studies on vitronectin in elastic tissue disorders, cutaneous amyloidosis, lichen ruber planus and porphyria. 245 88

Several biphosphonates (PCT) have been used as therapeutic agents for Paget's bone disease. CIPsMBP has recently been shown to have a significant antiosteoclastic activity while defect in mineralization was only apparent at high doses. For six months, we tested this drug in 23 pagetic patients distributed in three groups. Gr 1 (n = 5) receiving 200 mg/day showed a decrease of serum alkaline phosphatase (SAP) to 42 +/- 4% (p less than 0.01) of initial value (100%) while hydroxyprolinuria/creatinuria ratio (OH/Cr) dropped to 69 +/- 8% of baseline. In 4 patients receiving 400 mg/day, SAP improved to 48 +/- 9% of initial value (p less than 0.01) and OH/Cr to 40 +/- 3% (p less than 0.01). In the last group (n = 14) receiving 200 mg/day for 3 months, and 400 mg/day thereafter up to the 6th month SAP reduced to 53 +/- 4% and OH/Cr to 62 +/- 6% of initial value (p less than 0.01). Clinical improvement was significant from the first month of treatment. No resistance (mean decrease of SAP lower than 30%) was recorded and no radiological or clinical evidence of mineralization defect appeared. The clinical and biological tolerance was excellent throughout the study.
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PMID:(Chloro-4 phenyl) thiomethylene biphosphonate in Paget's bone disease. 260 91

We examined clinical and laboratory data and the liver pathology of 48 patients in whom porphyria cutanea tarda was related to alcohol ingestion, estrogen use and pregnancy, or was idiopathic. Biochemical test results, when abnormal, tended to be mild in most cases, with less than two-fold elevations of serum aminotransferases and alkaline phosphatase and mild hyperbilirubinemia. Fatty change, liver cell and Kupffer cell hemosiderosis and glycogenation of hepatocyte nuclei were frequent histologic findings in the 58 liver specimens studied. Alcoholic hepatitis, chronic hepatitis and cirrhosis were uncommon. Granuloma-like lobular aggregates consisting of iron- and ceroid-laden Kupffer cells, chronic inflammatory cells and fat droplets ("lobular lesions of porphyria cutanea tarda") were found in nearly two-thirds of specimens and appeared to be the most characteristic form of parenchymal damage in this form of porphyria. These lesions may be associated with pericentral fibrosis in alcoholic as well as estrogen-treated patients and may remit following therapeutic phlebotomy.
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PMID:Hepatic pathology in porphyria cutanea tarda. 619 67

A case report is presented of a young woman in whom symptomatic porphyria cutanea tarda (PCT) developed during copper chelation therapy for Wilson's disease. The 22 year old white woman was seen in the summer of 1978 because of development of blisters on the dorsa of the hands associated with focal atrophic hypopigmentation, generalized hyperpigmentation of the skin, and hpertrichosis of the lateral forehead and face. A sibling had died in childhood with Wilson's disease. When the patient developed hepatomegaly, ascites, and an acute hepatitis syndrome at the age of 11, penicillamine therapy was empirically started, with gradual symptomatic improvement. When evaluated at the age of 22, abnormal laboratory values included a total bilirubin of 1.2 mg%; alkaline phosphatase, 96 U; serum glutamic oxaloacetic transaminase (SGOT), 175 U; serum glutamic pyruvic transaminase (SGPT), 122 U; gamma glutamyl trans peptidase (GGTP), 64 U; and Bromsulphalein (BSP) retention, 21% at 45 minutes. Skin biopsy from the hand revealed a noninflammatory subepidermal bulla with prominently PAS positive vessel walls in the festooned dermal papillae at the base of the blister. A fragmented liver biopsy failed to reveal evidence of active hepatitis or cirrhosis, but considerable stainable iron was present in both hepatocytes and Kupffer cells. A rubeanic acid stain for copper was negative. The patient was diagnosed as having Wilson's disease, hepatic hemosiderosis, and PCT. Cessation of all ethanol consumption and discontinuation of the oral contraceptives which she had been taking for 6 years, was recommended. On examination 9 and 22 months after these modifications were instituted, the patient felt asymptomatic and was without evidence of any new blisters or scars of her skin. The hyperpigmentation and hypertrichosis persisted, but she rigidly adhered to a program of penicillamine, topical sunscreen application, and abnegation of alcohol. Liver function studies were normal, and urinary porphyrin levels returned toward normal values. The clinical onset of this patient's blistering disease was temporally associated with ethanol and exogenous estrogen medication.
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PMID:Porphyria cutanea tarda complicating Wilson's disease. 720 91

A 38-year-old black woman presented with a multisystem disease characterized by malaise, fever, sweats, and diffuse hyperpigmentation. Laboratory examinations showed anemia, elevated alkaline phosphatase, granulomas in the liver and bone marrow, and elevated porphyrins in the urine and feces, characteristic of porphyria cutanea tarda (PCT). A workup for an etiology of the granulomas was nonproductive, and the patient responded to systemic steroids.
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PMID:Porphyria cutanea tarda and sarcoidosis. 735 54

Porphyria cutanea tarda (PCT) is a condition that affects liver and skin by reduction of hepatic uroporphyrinogen decarboxylase activity. It is characterized by blistering lesions, erosions and crusts on sun-exposed areas. We report a 51-year-old male presenting with recurrent episodes of bullae, erosions, and crust on his neck and dorsum of the hands for 3 months. Aspartate aminotransferase, alanine aminotransferase, gamma-glutamyl transferase, alkaline phosphatase, lactate dehydrogenase levels, as well as total plasma porphyrin and urinary uroporphyrin levels were elevated. Based on the clinical manifestations, the history and laboratory findings, a diagnosis of PCT was made. The cutaneous and biochemical abnormalities of the patient improved with therapy of glycyrrhizin.
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PMID:A first report of porphyria cutanea tarda successfully treated with glycyrrhizin. 3126 8