FYH-eiwit, menselijk, recombinant
Kwaliteit
ISO Gecertificeerd
Levering
24-48 uur
Technische Specificaties
PHYH belongs to the family of iron (II) -dependent oxygenases, which typically incorporate one atom of dioxygen into the substrate and one atom into the succinate carboxylate group. PHYH is expressed in liver, kidney, and T-cells, but not in spleen, brain, heart, lung and skeletal muscle. It converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA. Defects in PHYH can cause Refsum disease (RD) . RD is an autosomal recessive disorder characterized clinically by a tetrad of abnormalities: retinitis pigmentosa, peripheral neuropathy, cerebellar ataxia, and elevated protein levels in the cerebrospinal fluid (CSF) . Patients exhibit accumulation of the branched-chain fatty acid, phytanic acid, in blood and tissues.
Ice Packs
-20°C
Beschrijving
FYH-eiwit, menselijk, recombinant Beschikbaar in 5 µg. Bestel eenvoudig online met snelle levering.
Specificaties
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