FLVCR1-1 transports heme from cytosol to extracellular region

Stable Identifier
R-HSA-917892
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
Export of heme to the blood by FLVCR
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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
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
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
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
heme transporter activity of FLVCR1-1 [plasma membrane]
Physical Entity
Activity
Orthologous Events
Authored
Reviewed
Created
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