Reactome | FLVCR1-1 transports heme from cytosol to extracellular region

FLVCR1-1 transports heme from cytosol to extracellular region

Stable Identifier

R-HSA-917892

Type

Reaction [transition]

Species

Homo sapiens

Compartment

plasma membrane

Synonyms

heme b(in) = heme b(out), Export of heme to the blood by FLVCR

ReviewStatus

5/5

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FLVCR1-1 transports heme from cytosol to extracellular region

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Heme is utilised as a prosthetic group in the production of hemoproteins inside cells. However, when intracellular heme accumulation occurs, heme is able to exert its pro-oxidant and cytotoxic action. The amount of free heme must be tightly controlled to maintain cellular homeostasis and avoid pathological conditions (Chiabrando et al. 2014). The heme transporter FLVCR is expressed in intestine and liver tissue, but also in developing erythroid cells where it is required to protect them from heme toxicity (Quigley et al, 2004; Rey et al, 2008). Two different isoforms have been described. FLVCR1-1 (FLVCR1a) resides in the plasma membrane and is responsible for heme detoxification in several cell types, such as erythroid progenitors, endothelial cells, hepatocytes, lymphocytes and intestinal cells.

Literature References

PubMed ID	Title	Journal	Year
15369674	Identification of a human heme exporter that is essential for erythropoiesis Quigley, JG, Yang, Z, Worthington, MT, Phillips, JD, Sabo, KM, Sabath, DE, Berg, CL, Sassa, S, Wood, BL, Abkowitz, JL	Cell	2004
18815190	Enhanced alternative splicing of the FLVCR1 gene in Diamond Blackfan anemia disrupts FLVCR1 expression and function that are critical for erythropoiesis Rey, MA, Duffy, SP, Brown, JK, Kennedy, JA, Dick, JE, Dror, Y, Tailor, CS	Haematologica	2008