Rare Diseases Symptoms Automatic Extraction

Hemoglobin α in the blood vessel wall.

[alpha-thalassemia]

Hemoglobin has been studied and well characterized in red blood cells for over 100 years. However, new work has indicated that the hemoglobin α subunit (Hbα) is also found within the blood vessel wall, where it appears to localize at the myoendothelial junction (MEJ) and plays a role in regulating nitric oxide (NO) signaling between endothelium and smooth muscle. This discovery has created a new paradigm for the control of endothelial nitric oxide synthase activity, nitric oxide diffusion, and, ultimately, vascular tone and blood pressure. This review discusses the current knowledge of hemoglobin׳s properties as a gas exchange molecule in the bloodstream and extrapolates the properties of Hbα biology to the MEJ signaling domain. Specifically, we propose that Hbα is present at the MEJ to regulate NO release and diffusion in a restricted physical space, which would have powerful implications for the regulation of blood flow in peripheral resistance arteries.

Diseases presenting "blood cells" symptom

  • adrenomyeloneuropathy
  • alpha-thalassemia
  • cadasil
  • cholangiocarcinoma
  • classical phenylketonuria
  • cutaneous mastocytosis
  • erdheim-chester disease
  • erythropoietic protoporphyria
  • heparin-induced thrombocytopenia
  • homocystinuria without methylmalonic aciduria
  • junctional epidermolysis bullosa
  • lamellar ichthyosis
  • lymphangioleiomyomatosis
  • monosomy 21
  • neonatal adrenoleukodystrophy
  • oral submucous fibrosis
  • scrub typhus
  • triple a syndrome
  • von hippel-lindau disease
  • waldenström macroglobulinemia
  • x-linked adrenoleukodystrophy
  • zellweger syndrome

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