Human feline leukemia virus subgroup C cellular receptor 1 ELISA Kit | FLVCR1 elisa kit
Human Feline leukemia virus subgroup C receptor-related protein 1, FLVCR1 ELISA Kit
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a member of the major facilitator superfamily of transporter proteins. The encoded protein is a heme transporter that may play a critical role in erythropoiesis by protecting developing erythroid cells from heme toxicity. This gene may play a role in posterior column ataxia with retinitis pigmentosa and the hematological disorder Diamond-Blackfan syndrome. [provided by RefSeq, Jan 2011]
Uniprot Description
FLVCR1: 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 FeLV-C in vitro. Defects in FLVCR1 are the cause of posterior column ataxia with retinitis pigmentosa (PCARP). 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. 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. Belongs to the major facilitator superfamily. Feline leukemia virus subgroup C receptor (TC 2.A.1.28.1) family.
Protein type: Membrane protein, integral; Transporter, ion channel; Transporter, iron; Membrane protein, multi-pass; Transporter
Chromosomal Location of Human Ortholog: 1q32.3
Cellular Component: mitochondrion; integral to plasma membrane; mitochondrial membrane; plasma membrane
Molecular Function: protein binding; transporter activity; heme transporter activity
Biological Process: spleen development; blood vessel development; heme transport; erythrocyte maturation; cellular iron ion homeostasis; in utero embryonic development; multicellular organismal development; multicellular organism growth; regulation of organ growth; mitochondrial transport; embryonic skeletal morphogenesis; transport; erythrocyte differentiation; embryonic digit morphogenesis; transmembrane transport
Disease: Posterior Column Ataxia With Retinitis Pigmentosa
Research Articles on FLVCR1
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Product Notes
The Human FLVCR1 flvcr1 (Catalog #AAA9331658) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9331658 ELISA Kit recognizes Human FLVCR1. It is sometimes possible for the material contained within the vial of "feline leukemia virus subgroup C cellular receptor 1, ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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