Rabbit anti-Mouse, Rat KIAA0196 Polyclonal Antibody | anti-KIAA0196 antibody

KIAA0196 Rabbit pAb

Cat. Num. AAA9143621

• Gene Names: KIAA0196; RTSC; SPG8
• Reactivity: Mouse, Rat
• Applications: Western Blot, Immunohistochemistry
• Purity: Affinity purification
• Synonyms: KIAA0196; Polyclonal Antibody; KIAA0196 Rabbit pAb; RTSC; SPG8; RTSC1; anti-KIAA0196 antibody

• Host: Rabbit
• Reactivity: Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Affinity purification
• Form/Format: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

• Applicable Applications for anti-KIAA0196 antibody: Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: WB: 1:500-1:2000; IHC: 1:50-1:200
• Immunogen: Recombinant fusion protein containing a sequence corresponding to amino acids 380-530 of human KIAA0196 (NP_055661.3).
• Positive Samples: Mouse thymus
• Preparation and Storage: Store at -20 degree C. Avoid freeze/thaw cycles.

Western Blot (WB)

(Western blot analysis of extracts of Mouse thymus, using KIAA0196 antibody at 1:1000 dilution.Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (AS014) at 1:10000 dilution.Lysates/proteins: 25ug per lane.Blocking buffer: 3% nonfat dry milk in TBST.Detection: ECL Enhanced Kit (RM00021).Exposure time: 300s.)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded rat brain using KIAA0196 Rabbit pAb at dilution of 1:100 (40x lens).Perform high pressure antigen retrieval with 10 mM citrate buffer pH 6.0 before commencing with IHC staining protocol.)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded mouse spinal cord using KIAA0196 Rabbit pAb at dilution of 1:100 (40x lens).Perform high pressure antigen retrieval with 10 mM citrate buffer pH 6.0 before commencing with IHC staining protocol.)

Related Product Information for anti-KIAA0196 antibody

Background: This gene encodes a 134 kDa protein named strumpellin that is predicted to have multiple transmembrane domains and a spectrin-repeat-containing domain. This ubiquitously expressed gene has its highest expression in skeletal muscle. The protein is named for Strumpell disease; a form of hereditary spastic paraplegia (HSP). Spastic paraplegias are a diverse group of disorders in which the autosomal dominant forms are characterized by progressive, lower extremity spasticity caused by axonal degeneration in the terminal portions of the longest descending and ascending corticospinal tracts. More than 30 loci (SPG1-33) have been implicated in hereditary spastic paraplegia diseases.

NCBI and Uniprot Product Information

• NCBI GI #: 120952851
• NCBI GeneID: 9897
• NCBI Accession #: NP_055661
• NCBI GenBank Nucleotide #: NM_014846.3
• UniProt Accession #: Q12768; Q3KQX5; Q8TBQ2; A8K4R7
• Molecular Weight: 134,286 Da
• NCBI Official Full Name: WASH complex subunit strumpellin
• NCBI Official Synonym Full Names: KIAA0196
• NCBI Official Symbol: KIAA0196
• NCBI Official Synonym Symbols: RTSC; SPG8
• NCBI Protein Information: WASH complex subunit strumpellin
• UniProt Protein Name: WASH complex subunit strumpellin
• Protein Family: WASH complex
• UniProt Gene Name: KIAA0196
• UniProt Entry Name: STRUM_HUMAN

NCBI Description

This gene encodes a 134 kDa protein named strumpellin that is predicted to have multiple transmembrane domains and a spectrin-repeat-containing domain. This ubiquitously expressed gene has its highest expression in skeletal muscle. The protein is named for Strumpell disease; a form of hereditary spastic paraplegia (HSP). Spastic paraplegias are a diverse group of disorders in which the autosomal dominant forms are characterized by progressive, lower extremity spasticity caused by axonal degeneration in the terminal portions of the longest descending and ascending corticospinal tracts. More than 30 loci (SPG1-33) have been implicated in hereditary spastic paraplegia diseases. [provided by RefSeq, Aug 2009]

Uniprot Description

KIAA0196: Component of the WASH complex, a complex present at the surface of endosomes that recruits and activates the Arp2/3 complex to induce actin polymerization. The WASH complex plays a key role in the fission of tubules that serve as transport intermediates during endosome sorting. Defects in KIAA0196 are the cause of spastic paraplegia autosomal dominant type 8 (SPG8). Spastic paraplegia is a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. 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. Belongs to the strumpellin family.

Chromosomal Location of Human Ortholog: 8q24.13

Cellular Component: endoplasmic reticulum; early endosome; cytosol

Molecular Function: protein binding

Biological Process: protein transport; endosome transport

Disease: Ritscher-schinzel Syndrome; Spastic Paraplegia 8, Autosomal Dominant