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Rabbit Anti-FLVCR/FITC Conjugated antibody
background:
FLVCR is responsible for the exportation of cytoplasmic heme groups. It is believed that it may protect developing erythroid cells from heme toxicity. Expression of FLVCR in cells will cause susceptibility to FeLSLVC (Feline leukemia virus subgroup C) in vitro. FLVCR is found in all hematopoietic tissues, including peripheral blood lymphocytes and fetal liver, and some expression is found in pancreas and kidney. It is down-regulated in haemopoietic progenitor cells undergoing differentiation and hemoglobinization.
Function:
Heme transporter that exports cytoplasmic heme. It can also export coproporphyrin and protoporphyrin IX, which are both intermediate products in the heme biosynthetic pathway. Does not export bilirubin. Heme export depends on the presence of HPX and may be required to protect developing erythroid cells from heme toxicity. Heme export also provides protection from heme or ferrous iron toxicities in liver and brain. Causes susceptibility to FeLSLVC in vitro.
Subunit:
Interacts with HPX.
Subcellular Location:
Cell membrane.
Tissue Specificity:
Found all hematopoietic tissues including peripheral blood lymphocytes. Some expression is found in pancreas and kidney.
DISEASE:
Defects in FLVCR1 are the cause of posterior column ataxia with retinitis pigmentosa (PCARP) [MIM:609033]. A neurodegenerative syndrome beginning in infancy with areflexia and retinitis pigmentosa. Nyctalopia (night blindness) and peripheral visual field loss are usually evident during late childhood or teenage years, with subsequent progressive constriction of the visual fields and loss of central retinal function over time. A sensory ataxia caused by degeneration of the posterior columns of the spinal cord results in a loss of proprioceptive sensation that is clinically evident in the second decade of life and gradually progresses. Scoliosis, camptodactyly, achalasia, gastrointestinal dysmotility, and a sensory peripheral neuropathy are variable features of the disease. Affected individuals have no clinical or radiological evidence of cerebral or cerebellar involvement. Note=Defective neuronal heme transmembrane export due to FLVCR1 mutations may abrogate the neuroprotective effects of neuroglobin and initiate an apoptotic cascade that results in the selective degeneration of photoreceptors in the neurosensory retina and sensory neurons in the posterior spinal cord.
Similarity:
Belongs to the major facilitator superfamily.
Feline leukemia virus subgroup C receptor (TC 2.A.1.28.1) family.
Database links:
Entrez Gene: 28982 Human
Omim: 609144 Human
SwissProt: Q9Y5Y0 Human
Unigene: 592816 Human
Unigene: 7055 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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