SAB1405987

Anti-CFI antibody produced in mouse

purified immunoglobulin, buffered aqueous solution

• Sinónimos: C3B-INA, FI, IF, KAF
• Código UNSPSC: 12352203
• NACRES: NA.41

Propiedades

• origen biológico: mouse
• Nivel de calidad: 100
• conjugado: unconjugated
• forma del anticuerpo: purified immunoglobulin
• tipo de anticuerpo: primary antibodies
• clon: polyclonal
• Formulario: buffered aqueous solution
• mol peso: antigen ~42.4 kDa
• reactividad de especies: human
• técnicas: western blot: 1 μg/mL
• Nº de acceso NCBI: BC020718.1
• Nº de acceso UniProt: P05156
• Condiciones de envío: dry ice
• temp. de almacenamiento: −20°C
• modificación del objetivo postraduccional: unmodified
• Información sobre el gen: human ... CFI(3426)

Descripción

Descripción general

This gene encodes a serine proteinase that is essential for regulating the complement cascade. The encoded preproprotein is cleaved to produce both heavy and light chains, which are linked by disulfide bonds to form a heterodimeric glycoprotein. This heterodimer can cleave and inactivate the complement components C4b and C3b, and it prevents the assembly of the C3 and C5 convertase enzymes. Defects in this gene cause complement factor I deficiency, an autosomal recessive disease associated with a susceptibility to pyogenic infections. Mutations in this gene have been associated with a predisposition to atypical hemolytic uraemic syndrome, a disease characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Primary glomerulonephritis with immmune deposits is another condition associated with mutation of this gene. (provided by RefSeq)

Inmunógeno

CFI (AAH20718.1, 1 a.a. ~ 377 a.a) full-length human protein.

Sequence
MKLLHVFLLFLCFHLRFCKVTYTSQEDLVEKKCLAKKYTHLSCDKVFCQPWQRCIEGTCVCKLPYQCPKNGTAVCATNRRSFPTYCQQKSLECLHPGTKFLNNGTCTAEGKFSVSLKHGNTDSEGIVEVKLVDQDKTMFICKSSWSMREANVACLDLGFQQGADTQRRFKLSDLSINSTECLHVHCRGLETSLAECTFTKRRTMGYQDFADVVCYTQKADSPMDDFFQCVNGKYISQMKACDGINDCGDQSDELCCKACQGKGFHCKSGVCIPSQYQCNGEVDCITGEDEVGCAGFASVAQEETEILTADMDAERRRIKSLLPKLSCGVKNRMHIRRKRIVGGKRAQLGKMKQISLDIFKGLHRVYAILEFCCIILK

Forma física

Solution in phosphate buffered saline, pH 7.4

Información de seguridad

• Código de clase de almacenamiento: 10 - Combustible liquids
• Clase de riesgo para el agua (WGK): WGK 1
• Punto de inflamabilidad (°F): Not applicable
• Punto de inflamabilidad (°C): Not applicable