Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UNIPROT:P20366 (substance P)
 21,176 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hepatic porphyrias are characterized by neurological symptoms manifested by abdominal pain, neuropathies and mental aberrations. Porphyrins are ubiquitous and essential biochemical constituents of living beings acting as mediators of oxidation reaction in the metabolism of the steroid, drugs, environmental chemicals or as a mean of exchanging gases, such as oxygen and carbon dioxide between the environment and the tissue of the body using endogenous polypeptide properties. The different porphyrins arising from the arrangement of normal heme synthesis are characterized by an accumulation and excretion of specific intermediate porphyrins and/or of precursors exerting toxic effect, initiating cascades of generations of polypeptides, neurotransmitters and gut-brain axis peptide responsible for the symptoms of clinical status. We studied polypeptide levels in 27 patients (19 females, 8 males) presenting acute attack of hepatic porphyria: 2 with ALA dehydratase-deficient porphyria; 9 with acute intermittent porphyria; 12 with porphyria cutanea tarda and 4 with variegate porphyria. During acute attacks of porphyria, polypeptides were found to be constantly increased: vasoactive intestinal polypeptide (VIP); neurotensin (NT); substance P; pancreatic polypeptide; gastrin-releasing peptide; gastrin and motilin. Administration of the somatostatin (antagonizing polypeptide), which was undetectable or low before treatment, apparently alleviated the acute symptomatology. Elevated levels of polypeptides, at least partly, contribute to appearance of acute symptoms in porphyria patients.
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PMID:Polypeptide levels increase during acute onset of hepatic porphyrias. 907 85

During acute attacks of hepatic porphyria, levels of polypeptides, vasoactive intestinal peptides, neurotensin, substance P, pancreatic polypeptide, gastrin releasing polypeptide, gastrin, and motilin increased in the circulation while the clinical symptoms were evident. However, somatostatin decrease was not detected. Somatostatin belongs to a group of regulatory peptides that antagonize the action of endogenous steroid hormones, and decreasing their bioavailability decreases the rate of synthesis of delta-aminolevulinate synthase, alpha-aminolevunilic acid (ALA), and polypeptides. Plasma exchange was conducted in courses for 2 consecutive days every 28 days (total of 6 courses), removing more than 100% of the patient plasma each time. Between the 2 courses of plasmapheresis, subcutaneous injections of somatostatin (100-500 mcg) were administered. A lasting disappearance of pain and complete remission were obtained in all 7 patients treated. Plasmapheresis combined with somatostatin may be considered as a treatment of porphyria exacerbation.
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PMID:Plasmapheresis combined with somatostatin is a successful treatment of porphyrias. 1022 63