Rabbit AFG3L2 Polyclonal Antibody | anti-AFG3L2 antibody

AFG3L2 Polyclonal Antibody

Cat. Num. AAA9457701

• Gene Names: AFG3L2; SCA28; SPAX5
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot, Immunohistochemistry, Immunofluorescence
• Purity: Affinity purification
• Synonyms: AFG3L2; Polyclonal Antibody; AFG3L2 Polyclonal Antibody; SCA28; SPAX5; anti-AFG3L2 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Affinity purification
• Form/Format: Supplied at 1.0mg/mL in phosphate buffered saline (without Mg2+ and Ca2+), pH7.4, 150mM NaCl, 0.02% sodium azide and 106% glycerol.; PBS with 0.02% sodium azide, 50% glycerol, pH7.59
• Concentration: 1mg/ml (varies by lot)

• Applicable Applications for anti-AFG3L2 antibody: Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)
• Application Notes: WB: 1:200-1:2000; IHC: 1:50-1:200; IF: 1:50-1:200
• Immunogen: Recombinant fusion protein of human AFG3L2 (NP_006787.2)
• Preparation and Storage: Store at -20 degree C. Avoid freeze/thaw cycles.

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded rat brain using AFG3L2 antibody at dilution of 1:200 .)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded human lung cancer using AFG3L2 antibody at dilution of 1:200 .)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded human colon using AFG3L2 antibody at dilution of 1:200 .)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded human appendix using AFG3L2 antibody at dilution of 1:200 .)

Western Blot (WB)

(Western blot analysis of extracts of various cell lines, using AFG3L2 antibody at 1:1000 dilution.)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded human breast cancer using AFG3L2 antibody at dilution of 1:200 .)

Related Product Information for anti-AFG3L2 antibody

This gene encodes a protein localized in mitochondria and closely related to paraplegin. The paraplegin gene is responsible for an autosomal recessive form of hereditary spastic paraplegia. This gene is a candidate gene for other hereditary spastic paraplegias or neurodegenerative disorders.

Product Categories/Family for anti-AFG3L2 antibody

Total protein Ab

NCBI and Uniprot Product Information

• NCBI GI #: 300192933
• NCBI GeneID: 10939
• NCBI Accession #: NP_006787.2
• NCBI GenBank Nucleotide #: NM_006796.2
• UniProt Accession #: Q9Y4W6; Q6P1L0
• Molecular Weight: 88,584 Da
• NCBI Official Full Name: AFG3-like protein 2
• NCBI Official Synonym Full Names: AFG3 like matrix AAA peptidase subunit 2
• NCBI Official Symbol: AFG3L2
• NCBI Official Synonym Symbols: SCA28; SPAX5
• NCBI Protein Information: AFG3-like protein 2
• UniProt Protein Name: AFG3-like protein 2
• Protein Family: AFG3-like protein
• UniProt Gene Name: AFG3L2
• UniProt Entry Name: AFG32_HUMAN

NCBI Description

This gene encodes a protein localized in mitochondria and closely related to paraplegin. The paraplegin gene is responsible for an autosomal recessive form of hereditary spastic paraplegia. This gene is a candidate gene for other hereditary spastic paraplegias or neurodegenerative disorders. [provided by RefSeq, Jul 2008]

Uniprot Description

AFG3L2: ATP-dependent protease which is essential for axonal development. Defects in AFG3L2 are the cause of spinocerebellar ataxia type 28 (SCA28). It is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA28 is an autosomal dominant cerebellar ataxia (ADCA) with a slow progressive course and no evidence of sensory involvement or cognitive impairment. Defects in AFG3L2 are the cause of spastic ataxia autosomal recessive type 5 (SPAX5). A neurodegenerative disorder characterized by early onset spasticity, peripheral neuropathy, ptosis, oculomotor apraxia, dystonia, cerebellar atrophy, and progressive myoclonic epilepsy.

Protein type: EC 3.4.24.-; Cell development/differentiation; Membrane protein, integral; Mitochondrial; Protease; Membrane protein, multi-pass; Chaperone

Chromosomal Location of Human Ortholog: 18p11

Cellular Component: mitochondrial inner membrane; mitochondrion

Molecular Function: ATP-dependent peptidase activity; metallopeptidase activity; protein binding; unfolded protein binding

Biological Process: cristae formation; mitochondrial fusion; nerve development; protein complex assembly; protein import into mitochondrial intermembrane space

Disease: Spastic Ataxia 5, Autosomal Recessive; Spinocerebellar Ataxia 28