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Invitrogen™ Bestrophin 1 Polyclonal Antibody
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Catalog No. PIPA589606
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PIPA589606 100 μL
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Catalog No. PIPA589606 Supplier Invitrogen™ Supplier No. PA589606
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Rabbit Polyclonal Antibody

Immunogen sequence: QPNQEDEEDA HAGIIGRFLG LQSHDHHPPR ANSRTKLLWP KRESLLHEGL PKNHKAAKQN VRGQEDNKAW KLKAVDAFKS APLYQRPGYY SAPQTPLSPT PMFFPLEPSA PSKLHSVTGI DTKDKSLKTV SSGAKKSFEL LSESDGALME HPEVSQVRRK TVEFNLTDMP EIPENHLKEP LEQSPTNIHT TLKDHMDPYW ALENRDEAHS; Positive Samples: Mouse liver, Mouse lung; Cellular Location: Basolateral cell membrane, Cell membrane, Multi-pass membrane protein.

The retinal pigment epithelium (RPE) and choroid represent a differentiated system of the eye that sustains normal retinal health and function. Best vitelliform macular dystrophy, known as Best disease, is an early-onset autosomal dominant condition in which accumulation of lipofuscin-like material within and beneath the RPE leads to progressive loss of central vision. The lipofuscin-like material in the macular area appears as a yellow mass like the yolk of an egg that later becomes darker and irregular in color, a process known as scrambling the egg. Best disease is frequently a reflection of mutations in the Bestrophin gene, which encodes a protein containing four putative transmembrane domains and localizes to the basolateral plasma membrane of RPE cells. Human Bestrophin forms oligomeric chloride channels that are sensitive to intracellular calcium. Missense mutations at the Bestrophin locus reduces or abolishes Bestrophin protein mediated membrane current. The human Bestrophin gene encodes a 585 amino acid protein.
TRUSTED_SUSTAINABILITY

Specifications

Antigen Bestrophin 1
Applications ELISA, Western Blot
Classification Polyclonal
Concentration 0.25 mg/mL
Conjugate Unconjugated
Formulation PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
Gene BEST1
Gene Accession No. O76090, O88870
Gene Alias ARB; BEST; BEST 1; Best disease; best macular dystrophy; BEST1; Best1V1Delta2; bestrophin; bestrophin 1; Bestrophin1; Bestrophin-1; BMD; Bmd1; LOW QUALITY PROTEIN: bestrophin-1; mBest1; RP50; TU15B; vitelliform macular dystrophy (Best disease, bestrophin); vitelliform macular dystrophy 2 homolog; vitelliform macular dystrophy protein 2; Vitelliform macular dystrophy protein 2 homolog; VMD 2; Vmd2
Gene Symbols BEST1
Host Species Rabbit
Immunogen Recombinant fusion protein containing a sequence corresponding to amino acids 376-585 of human BEST1 (NP_0041741).
Purification Method Affinity chromatography
Quantity 100 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 24115, 7439
Target Species Human, Mouse
Content And Storage -20°C, Avoid Freeze/Thaw Cycles
Product Type Antibody
Form Liquid
Isotype IgG
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WARNING: Cancer - www.P65Warnings.ca.gov
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