Rabbit Anti-Nebulette/FITC Conjugated antibody

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Actin binding Z disk protein; bA56H7.1; LIM nebulette; LNEBL; NEBL; NEBL_HUMAN.

Cat:

SL9468R-FITC

Species Reactivity：

Mouse,(predicted: Human,Rat,)

Immunogen：

KLH conjugated synthetic peptide derived from human Nebulette

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：

116kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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background:
Nebulette binds to actin and plays an important role in Z-disk assembly. It is a cardiac homolog of nebulin and its expression is abundant in cardiac muscle. Nebulette defects are assocated with non-familial idiopathic cardiacmyopathy.
Nebulin and nebulette are homologous thin-filament associated proteins expressed in skeletal and cardiac muscles, respectively. Nebulette, unlike nebulin, is confined to the Z-disc region of the sarcomere and does not span the whole thin filament length. Nebulette colocalizes with α-actinin in the pre-, nascent, and mature myofibrils. A polymorphism in the actin-binding motif of nebulette is a genetic marker of susceptibility to nonfamilial idiopathic dilated cardiomyopathy (IDC), characterized by a thin-walled heart and systolic dysfunction.

Function:
Binds to actin and plays an important role in the assembly of the Z-disk. Isoform 2 might play a role in the assembly of focal adhesion.

Subunit:
Isoform 2 interacts with ZYX (Zyxin).

Subcellular Location:
Cytoplasmic.

Tissue Specificity:
Abundantly expressed in cardiac muscle, but not in skeletal or smooth muscle. Localized to Z-lines in cardiac cells and to dense bodies in nonmuscle cells. Isoform 2 is expressed in non-muscle cells such as in fibroblasts.

Similarity:
Contains 23 nebulin repeats.
Contains 1 SH3 domain.

Database links:

Entrez Gene: 10529 Human

Entrez Gene: 74103 Mouse

Omim: 605491 Human

SwissProt: O76041 Human

SwissProt: Q9DC07 Mouse

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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