Pharmacology of the Porphyrins and Porphobilinogen

Goldberg, A.; Patón, W. D. M.; Thompson, J. W.

doi:10.1111/j.1476-5381.1954.tb00822.x

Cited by 26 publications

(8 citation statements)

References 11 publications

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“…There would be more of a tendency to polymerize at higher concentrations, and the products formed may have artifactually elevated [3H]NE uptake by causing membrane damage. Hematoporphyrin has been shown to be toxic to platelet membranes in vitro (27 (28,29). In nephrectomized rats receiving ALA or PBG intravenously, plasma concentrations of >5 mg/100 ml have been achieved without any alteration in blood pressure or symptoms of nervous system toxicity (23,30).…”

Section: Discussionmentioning

confidence: 99%

Catecholamine Uptake, Accumulation, and Release in Acute Porphyria

Beal¹,

Atuk²,

Westfall³

et al. 1977

J. Clin. Invest.

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A B S T R A C T Hypertension and tachycardia are well known features of acute porphyria and have been shown to be related to increased circulating catecholamines. The mechanism by which circulating catecholamines are increased was studied using the isolated perfused rat heart and human platelets as a model of adrenergic neuronal function. It was found that neither 8-aminolevulinate (ALA) nor porphobilinogen (PBG) blocked uptake or caused release in the isolated perfused rat heart. Platelets from six patients with acute prophyria, three in remission and three latent, with matching normal controls were studied with regard to their uptake of [3H]

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Section: Discussionmentioning

confidence: 99%

Catecholamine Uptake, Accumulation, and Release in Acute Porphyria

Beal¹,

Atuk²,

Westfall³

et al. 1977

J. Clin. Invest.

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“…Recently, the amount of free erythrocyte protoporphyrins (FEP) of renal dialysis patients has been shown to correlate with decreases in their motor nerve conduction velocities.8 T h e accumulation of porphyrins themselves has been shown to be nontoxic. 5 It has been suggested that patients with sickle cell anemia have somewhat elevated FEP levels as a result of their hemoglobinopathy. However, a random assessment of 27 SSA patients in our clinic failed to demonstrate elevated FEP in the absence of elevated blood lead levels.…”

Section: Discussionmentioning

confidence: 99%

Peripheral neuropathy in lead‐intoxicated sickle cell patients

et al. 1978

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Peripheral neuropathy and hypertension caused by lead intoxication are reported in two children with sickle cell anemia. One child had generalized weakness in the initial occurrence and distal paralysis during a relapse two years later. The second child had foot and wrist drop. Both had slow peripheral nerve conduction velocities during the episodes. Chelation therapy was successful and resulted in a return of strength (over a period of several months) and a normalization of the blood pressures. Children with sickle cell anemia who are subjected to lead intoxication appear to be predisposed to peripheral nerve damage.

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“…In the past the demonstration that ALA and PBG are pharmacologically inert (Goldberg, Paton and Thompson, 1954) has led to suggestions that there is a deficiency of a substance required to protect nerve function, or of pyridoxine (Ridley, 1969), or that the abnormality of porphyrin precursor metabolism is merely a spectacular side effect of a more fundamental metabolic disturbance (Neuberger, 1968) possibly involving oxidation of NADH (Tschudy and Bonkowski, 1972). Recently Becker, Viljoen, and Kramer (1971) The porphyrias: a review that ALA inhibits brain tissue ATPase and have suggested that in the acute phase of porphyria ALA is taken up by nerve tissue and causes paralysis of conduction by inhibition of the Na+-K+-dependent ATPase, while Feldman, Levere, Lieberman, Cardinal, and Watson (1971) have reported that PBG in concentrations similar to those found in the cerebrospinal fluid during acute attacks, and one of its non-enzymically produced condensation products porphobilin, produce presynaptic neuromuscular inhibition.…”

Section: The Nature Of the Metabolic Abnormality In The Inherited Hepmentioning

confidence: 99%