CFHR1

CFHR1

Target · Complement regulator

CFHR1 Complement factor H related 1 · Complement factor H-related protein 1

CFHR1 (complement factor H related 1, also known as FHR1, H36-1 and CFHL1) is a secreted member of the complement factor H protein family. It is involved in complement regulation and can associate with lipoproteins. Browse Assay Genie's current range of CFHR1 research reagents below, or read on for the biology behind the target.

Secreted proteinComplement regulatorLinked to aHUS risk
330 aa
Protein length
~38 kDa
Molecular weight
1q31.3
Gene locus
Sushi domain
Protein family
Target information

What is CFHR1 (complement factor H related 1)?

CFHR1 is involved in complement regulation. Its dimerised forms have avidity for tissue-bound complement fragments and compete efficiently with the physiological complement inhibitor CFH (complement factor H). This competition places the CFHR1 protein within the balance of complement control at tissue surfaces. CFHR1 can also associate with lipoproteins, and it may play a role in lipid metabolism.

The CFHR1 gene encodes a secreted protein that belongs to the complement factor H protein family. HGNC classifies it within the sushi domain containing group and among the complement system regulators and receptors. The protein is also known as FHR1, H36-1, H36-2, HFL1, HFL2, CFHL and CFHL1, and the gene has previously been listed under pseudogene symbols such as CFHR1P and CFHL1P, reflecting earlier names such as H factor (complement)-like 1.

NCBI Gene reports that CFHR1 binds Pseudomonas aeruginosa elongation factor Tuf together with plasminogen, which is proteolytically activated. It has been proposed that Tuf acts as a virulence factor by acquiring host proteins to the pathogen surface, controlling complement and facilitating tissue invasion. Mutations in the CFHR1 gene are associated with an increased risk of atypical hemolytic-uremic syndrome, which makes the protein a point of interest in studies of complement-mediated disease.

Complement regulation

CFHR1 is involved in complement regulation and competes with the physiological inhibitor CFH for tissue-bound complement fragments.

Atypical haemolytic-uraemic syndrome

Mutations in the CFHR1 gene are associated with an increased risk of atypical hemolytic-uremic syndrome.

Host-pathogen interaction

CFHR1 binds Pseudomonas aeruginosa elongation factor Tuf together with plasminogen, a mechanism proposed to help the pathogen control complement.

Lipid metabolism research

CFHR1 can associate with lipoproteins and may play a role in lipid metabolism.

Background

CFHR1 frequently asked questions

What does CFHR1 do?

CFHR1 is involved in complement regulation. Its dimerised forms have avidity for tissue-bound complement fragments and efficiently compete with the complement inhibitor CFH. It can also associate with lipoproteins and may play a role in lipid metabolism. Check the datasheet of any product for its validated reactivity.

What other names does CFHR1 go by?

CFHR1 is also called FHR1, H36-1, H36-2, HFL1, HFL2, CFHL and CFHL1. Older symbols include CFHR1P and CFHL1P. Previous names include H factor (complement)-like 1, H factor (complement)-like 2 and complement factor H-related 1 pseudogene.

Is CFHR1 linked to disease?

According to NCBI Gene, mutations in the CFHR1 gene are associated with an increased risk of atypical hemolytic-uremic syndrome. The protein also binds Pseudomonas aeruginosa elongation factor Tuf with plasminogen, which may help the pathogen control complement.

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Related targets

Other members of the complement factor H protein family and related complement regulators with products at Assay Genie.

Gene and protein annotations from HGNC, NCBI Gene and UniProt Q03591; product specifications from Assay Genie datasheets.

CFHR1 Product listing