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CFHR1

CFHR1 is a secreted protein belonging to the complement factor H protein family.The human complement factor H protein family consists of the complement and immune regulators factor H, the factor H-like protein 1(FHL-1) and five factor H-related proteins (CFHR-1 to -5). Members of the H-related protein family are exclusively composed of individually folded protein domains, termed short consensus repeats (SCRs) or complement control modules. CFHR1 binds to Pseudomonas aeruginosa elongation factor Tuf together with plasminogen, which is proteolytically activated. CFHR1 might be involved in complement regulation. It can associate with lipoproteins and may play a role in lipid metabolism.

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CFHR1 Related Products

CFHR1 Proteins

CFHR1 Antibodies

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CFHR1 ELISA Pair sets

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CFHR1 cDNA Clones

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CFHR1 Related Areas

 

CFHR1 Related Pathways

CFHR1 Alternative Names

CFHL, CFHL1, CFHL1P, CFHR1P, FHR1, H36-1, H36-2, HFL1, HFL2, FHR-1, H factor (complement)-like 1, H factor (complement)-like 2, H-factor-like 1, H36, complement factor H-related 1 pseudogene, complement factor H-related protein 1, h factor-like protein 1 [Homo sapiens]

AI194696, CFHRB, Cfhl1, complement component factor h-like 1, complement factor H-related protein [Mus musculus]

Summaries for CFHR1

Entrez Gene summary for CFHR1:

This CFHR1 gene encodes a secreted protein belonging to the complement factor H protein family. CFHR1 binds to Pseudomonas aeruginosa elongation factor Tuf together with plasminogen, which is proteolytically activated. It is proposed that Tuf acts as a virulence factor by acquiring host proteins to the pathogen surface, controlling complement, and facilitating tissue invasion. Mutations in this CFHR1 gene are associated with an increased risk of atypical hemolytic-uremic syndrome. [provided by RefSeq, Oct 2009]

Wikipedia summary for CFHR1:

Complement factor H-related protein 1 is a protein that in humans is encoded by the CFHR1 gene.

Human CFHR1 Protein General Information

 

Protein names

Complement factor H-related protein 1, Short name=FHR-1

Sequence length

330 AA.

Sequence similarities:

CFHR1 contains 5 Sushi (CCP/SCR) domains.

Post-translational modification:

N-glycosylated. Two forms are observed; one with a single side chain and the other with two.

Subcellular location: Secreted.
Tissue specificity

CFHR1 is expressed by the liver and secreted in plasma.

General information above from UniProt

Function for CFHR1 Protein

UniProtKB:

CFHR1 might be involved in complement regulation. CFHR1 can associate with lipoproteins and may play a role in lipid metabolism.

Genatlas:

  • CFHR1 is involved in complement regulation
  • CFHR1 can associate with lipoproteins and may play a role in lipid metabolism
  • CFHR1 represents a complement regulator that acts downstream of factor H by inhibiting the C5 convertase and the assembly and membrane insertion of the terminal complement complex (TCC)
  • CFHR3 and CFHR1 inhibit C5a generation and complement-mediated chemotaxis of neutrophils
  • CFHR1 inhibits the C5 convertase and the terminal complement pathway
  • CFHR1 may be involved in the regulation of complement during synapse elimination, and were found to be deleted in a Z-RTT (Rett milder Zappella variant) but duplicated in two classic patients

Homology for human CFHR1

Phenotype Information for CFHR1

Gene/Locus Phenotype
CFHR1, FHR1, HFL1, CFHL1 {Hemolytic uremic syndrome, atypical, susceptibility to}
{Macular degeneration, age-related, reduced risk of}

Phenotype Information for CFHR1 from OMIM (Online Mendelian Inheritance in Man)

Drugs for CFHR1

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