BFSP1 Antibody

Datasheet

Code	CSB-PA002680GA01HU
Size	$600
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Product Details
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Target Background

Product Details

Uniprot No.

Q12934

Target Names

BFSP1

Alternative Names

Beaded filament structural protein 1 antibody; Beaded filament structural protein 1 filensin antibody; BFSP1 antibody; BFSP1_HUMAN antibody; CP115 antibody; CP94 antibody; CP95 antibody; Cytoskeletal protein; 115 KD antibody; Filensin antibody; Lens fiber cell beaded filament structural protein CP 115 antibody; Lens fiber cell beaded-filament structural protein CP 115 antibody; Lens intermediate filament like heavy antibody; Lens intermediate filament-like heavy antibody; LIFL H antibody; LIFL-H antibody

Raised in

Rabbit

Species Reactivity

Human

Immunogen

Human BFSP1

Immunogen Species

Homo sapiens (Human)

Isotype

IgG

Purification Method

Antigen Affinity purified

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.

Tested Applications

ELISA,WB,IF

Protocols

ELISA Protocol
Western Blotting(WB) Protocol
Immunofluorescence (IF) Protocol

Troubleshooting and FAQs

Antibody FAQs

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Lead Time

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Usage

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

Customer Reviews and Q&A

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Target Background

Function

Required for the correct formation of lens intermediate filaments as part of a complex composed of BFSP1, BFSP2 and CRYAA. Involved in altering the calcium regulation of MIP water permeability.

Gene References into Functions

The results suggest that the N-terminal domain of CRYAA is required during in vitro complex formation with filensin and phakinin. PMID: 28935373
A novel mutation (c.1042G>A) at exon 7 of BFSP1, which creates a substitution of an aspartate to an asparagine (p.D348N) was identified to be associated with autosomal dominant congenital cataract in a Chinese family. PMID: 24379646
The crystallin beta cluster on chromosome 22, GJA3, and BFSP1 play a major role in maintaining lens transparency. PMID: 24319337
This is the first report of a mutation in the BFSP1 gene associated with human inherited cataracts. PMID: 17225135

Involvement in disease

Cataract 33, multiple types (CTRCT33)

Subcellular Location

Cell membrane; Peripheral membrane protein; Cytoplasmic side. Cytoplasm. Cytoplasm, cytoskeleton. Cytoplasm, cell cortex.

Protein Families

Intermediate filament family

Tissue Specificity

Expressed in the cortex and nucleus of the retina lens (at protein level).

Database Links

HGNC: 1040

OMIM: 603307

KEGG: hsa:631

STRING: 9606.ENSP00000367104

UniGene: Hs.129702

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Service

Phage Display Service

Antibody Service

Protein Service

Gene Synthesis Service

Oligo Synthesis Service

Antibody FAQs What Is an Antibody? How to Choose an Antibody for Scientific Research? The difference between primary antibody and secondary antibod The Meaning of Irregular Antibody Screening Tag Antibodies, Your Good Partner in Protein Research How to Choose the Loading Control Antibodies?

Call us

301-363-4651 (Available 9 a.m. to 5 p.m. CST from Monday to Friday)

[email protected]

Address

7505 Fannin St., Ste 610, Room 7 (CUBIO Innovation Center), Houston, TX 77054, USA

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