Essais Alpha B Crystallin

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Alpha B Crystallin est un gène codé par le symbole CRYAB. D'autres noms incluent: Alpha-crystallin B chain; Alpha(B)-crystallin; Heat shock protein beta-5; HspB5; Renal carcinoma antigen NY-REN-27; Rosenthal fiber component; CRYAB; CRYA2; HSPB5. Alpha B Crystallin a une masse de 20.16kDa, une longueur d'acide aminé de 175, et est impliqué dans les maladies: Myopathy, myofibrillar, 2; Cataract 16, multiple types; Myopathy, myofibrillar, fatal infantile hypertonic, alpha-B crystallin-related; Cardiomyopathy, dilated 1II.

Nous proposons 7 Alpha B Crystallin kits ELISA pour la détection qualitative ou quantitative de Alpha B Crystallin à partir d'échantillons Humain, Souris et Rat.

Informations sur les Gènes et les Protéines

Résumé UniProt
May contribute to the transparency and refractive index of the lens. Has chaperone-like activity, preventing aggregation of various proteins under a wide range of stress conditions.
Résumé Entrez
Mammalian lens crystallins are divided into alpha, beta, and gamma families. Alpha crystallins are composed of two gene products: alpha-A and alpha-B, for acidic and basic, respectively. Alpha crystallins can be induced by heat shock and are members of the small heat shock protein (HSP20) family. They act as molecular chaperones although they do not renature proteins and release them in the fashion of a true chaperone; instead they hold them in large soluble aggregates. Post-translational modifications decrease the ability to chaperone. These heterogeneous aggregates consist of 30-40 subunits; the alpha-A and alpha-B subunits have a 3:1 ratio, respectively. Two additional functions of alpha crystallins are an autokinase activity and participation in the intracellular architecture. The encoded protein has been identified as a moonlighting protein based on its ability to perform mechanistically distinct functions. Alpha-A and alpha-B gene products are differentially expressed; alpha-A is preferentially restricted to the lens and alpha-B is expressed widely in many tissues and organs. Elevated expression of alpha-B crystallin occurs in many neurological diseases; a missense mutation cosegregated in a family with a desmin-related myopathy. Alternative splicing results in multiple transcript variants.
Spécificité tissulaire
Lens as well as other tissues (PubMed:838078, PubMed:2387586). Expressed in myocardial tissue (PubMed:28493373).
Implication dans la maladie
Myopathy, myofibrillar, 2: A form of myofibrillar myopathy, a group of chronic neuromuscular disorders characterized at ultrastructural level by disintegration of the sarcomeric Z disk and myofibrils, and replacement of the normal myofibrillar markings by small dense granules, or larger hyaline masses, or amorphous material. MFM2 is characterized by weakness of the proximal and distal limb muscles, weakness of the neck, velopharynx and trunk muscles, hypertrophic cardiomyopathy, and cataract in a subset of patients.

Cataract 16, multiple types: 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. CTRCT16 includes posterior polar cataract, among others. Posterior polar cataract is a subcapsular opacity, usually disk-shaped, located at the back of the lens.

Myopathy, myofibrillar, fatal infantile hypertonic, alpha-B crystallin-related: A form of myofibrillar myopathy, a group of chronic neuromuscular disorders characterized at ultrastructural level by disintegration of the sarcomeric Z disk and myofibrils, and replacement of the normal myofibrillar markings by small dense granules, or larger hyaline masses, or amorphous material. MFMFIH-CRYAB has onset in the first weeks of life after a normal neonatal period. Affected infants show rapidly progressive muscular rigidity of the trunk and limbs associated with increasing respiratory difficulty resulting in death before age 3 years.

Cardiomyopathy, dilated 1II: A disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.
Similitudes de séquence
Belongs to the small heat shock protein (HSP20) family.
Localisation cellulaire
Cytoplasm. Nucleus.

Translocates to the nucleus during heat shock and resides in sub-nuclear structures known as SC35 speckles or nuclear splicing speckles (PubMed:19464326). Localizes at the Z-bands and the intercalated disk in cardiomyocytes (PubMed:28493373).
Standard Curve - Human alpha B Crystallin ELISA Kit (A76373) - Antibodies.com
Standard Curve - Mouse alpha B Crystallin ELISA Kit (A4977) - Antibodies.com
Standard Curve - Human Alpha B Crystallin ELISA Kit (A313927) - Antibodies.com
Voir le roduitELISA de 90 minutes
Standard Curve - Mouse alpha B Crystallin ELISA Kit (A76372) - Antibodies.com
Standard Curve - Human alpha B Crystallin ELISA Kit (A4978) - Antibodies.com
Western Blot - CRYAB (phospho Ser19) Cell Based ELISA Kit (CBP1358) - Antibodies.com
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Western Blot - CRYAB (phospho Ser45) Cell Based ELISA Kit (CBP1359) - Antibodies.com
(4)

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