Rabbit Anti-SFSP2/FITC Conjugated antibody

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49 kDa cytoskeletal protein; Beaded filament protein CP49; Beaded filament structural protein 2; Beaded filament structural protein 2, phakinin; Bfps2, Cytoskeletal protein, 49 kD; BFSP2; BFSP2_HUMAN; CP47; CP49; Lens fiber cell beaded filament protein CP

Cat:

SL11015R-FITC

Species Reactivity：

(predicted: Human,Mouse,Rat,Dog,)

Immunogen：

KLH conjugated synthetic peptide derived from human BFSP2/Phakinin

Format：

Lyophilized or Liquid

Storage instructions：

Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

46kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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background:
Phakinin is a membrane-associated and cytoskeletal intermediate filament (IF) protein specific to the eye lens. IFs are cytoskeletal structures that typically contain a head, rod and tail domain. Unlike most IFs, Phakinin completely lacks the SLCterminal tail domain thus contributing to the unique structure of the beaded filament that is specific to the lens. Phakinin is required for the assembly of beaded filaments and cytoskeletal networks that are important for the long-term maintenance of optical properties and transparency of the lens. Phakinin copolymerizes with Filensin, another IF protein, to form the 10-nm filamentous structures of the beaded filaments. Phakinin is also capable of self-assembling into filament-like structures that form thicker bundles. Mutations in the gene encoding Phakinin can result in lens cataract.

Function:
Involved in stabilization of lens fiber cell cytoskeleton.

Subunit:
Associates with SFSP1. Interacts with LGSN.

Subcellular Location:
Membrane. Cytoplasm. Cytoplasm, cytoskeleton. Membrane- and cytoskeleton-associated.

Tissue Specificity:
Lens.

DISEASE:
Defects in SFSP2 are the cause of cataract autosomal dominant SFSP2-related (ADSLCSFSP2); also known as cataract autosomal dominant multiple types 1. Cataract is an opacification of the crystalline lens of the eye that frequently results in visual impairment or blindness. Opacities vary in morphology, are often confined to a portion of the lens, and may be static or progressive. In general, the more posteriorly located and dense an opacity, the greater the impact on visual function. Cataract autosomal dominant SFSP2-related is characterized by a variable phenotype that may or may not be consistent within a family. The opacities can be nuclear, sutural, stellate cortical, lamellar, cortical, nuclear embryonic, Y-sutural, punctate cortical, congenital or with juvenile- and adult-onset.

Similarity:
Belongs to the intermediate filament family.

Database links:

Entrez Gene: 8419 Human

Omim: 603212 Human

SwissProt: Q13515 Human

Unigene: 659862 Human

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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