ALS5; CMT2X; KIAA1840

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

Polyclonal

IgG

Rabbit

Affinity Purification

Store at -20 degree C. Avoid freeze/thaw cycles.

Small volumes of anti-SPG11 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

The protein encoded by this gene is a potential transmembrane protein that is phosphorylated upon DNA damage. Defects in this gene are a cause of spastic paraplegia type 11 (SPG11). Multiple transcript variants encoding different isoforms have been found for this gene.

Primary antibody

Western Blot (WB)

WB: 1:200 - 1:2000

Western blot analysis of extracts of U-87MG cells, using SPG11 antibody at 1:1000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Enhanced Kit.
Exposure time: 90s.

279kDa

SPG11, spatacsin vesicle trafficking associated

spatacsin

Spatacsin

KIAA1840??[Similar Products]

The protein encoded by this gene is a potential transmembrane protein that is phosphorylated upon DNA damage. Defects in this gene are a cause of spastic paraplegia type 11 (SPG11). Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, May 2009]

SPG11: Defects in SPG11 are the cause of spastic paraplegia autosomal recessive type 11 (SPG11). Spastic paraplegia is a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, integral; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: 15q21.1

Cellular Component: cytoplasm; cytoplasmic vesicle; cytosol; lysosomal membrane; nucleolus; plasma membrane; synapse

Molecular Function: protein binding

Biological Process: axon cargo transport; synaptic transmission; synaptic vesicle transport

Disease: Amyotrophic Lateral Sclerosis 5, Juvenile; Charcot-marie-tooth Disease, Axonal, Type 2x; Spastic Paraplegia 11, Autosomal Recessive

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