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Mouse Anti-Tor1aip1 Recombinant Antibody (RL13) (CBMAB-L2972-YJ)

Summary

Host Animal
Mouse
Specificity
Mouse, Rat
Clone
RL13
Antibody Isotype
IgG1
Application
WB, IHC, ICC, IF, IHC-P

Basic Information

Immunogen
Pore complex-lamina fraction isolated from rat liver nuclear envelopes.
Specificity
Mouse, Rat
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Buffer
PBS, pH 7.4
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
torsin A interacting protein 1
Introduction
Torsin A interacting protein 1 is required for nuclear membrane integrity. It induces TOR1A and TOR1B ATPase activity and is required for their location on the nuclear membrane. It binds to A- and B-type lamins. It is a possible role in membrane attachment and assembly of the nuclear lamina.
Entrez Gene ID
Mouse208263
Rat246314
UniProt ID
MouseQ921T2
RatQ5PQX1
Alternative Names
LAP1; Lap1B; torsin-1A-interacting protein 1; lamina-associated polypeptide 1B
Function
Required for nuclear membrane integrity. Induces TOR1A and TOR1B ATPase activity and is required for their location on the nuclear membrane. Binds to A- and B-type lamins. Possible role in membrane attachment and assembly of the nuclear lamina.
Biological Process
Biological Process membrane organization Source:GO_Central1 Publication
Biological Process nuclear membrane organization Source:GO_Central1 Publication
Biological Process positive regulation of ATP-dependent activity Source:UniProtKB1 Publication
Biological Process protein localization to nuclear envelope Source:Ensembl
Biological Process protein localization to nucleus Source:UniProtKB
Cellular Location
Nucleus inner membrane
Involvement in disease
Myopathy, autosomal recessive, with rigid spine and distal joint contractures (MRRSDC):
An autosomal recessive degenerative myopathy characterized by muscle weakness initially involving the proximal lower limbs, followed by distal upper and lower limb muscle weakness and atrophy. Other features include joint contractures, rigid spine, and restricted pulmonary function. Cardiac involvement has been observed in some patients. Disease onset is in the first or second decades of life.
Topology
Nuclear: 1-338
Helical: 339-355
Perinuclear space: 356-583
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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