Rabbit anti-Human AFG3L2 Polyclonal Antibody | anti-AFG3L2 antibody

AFG3L2, NT (AFG3L2, AFG3-like protein 2, Paraplegin-like protein)

Cat. Num. AAA645153

• Gene Names: AFG3L2; SCA28; SPAX5
• Reactivity: Human
• Applications: ELISA, Western Blot, Immunohistochemistry
• Purity: Affinity Purified; Purified by Protein A affinity chromatography.
• Synonyms: AFG3L2; Polyclonal Antibody; NT (AFG3L2; AFG3-like protein 2; Paraplegin-like protein); Anti -AFG3L2; anti-AFG3L2 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Human
• Purity/Purification: Affinity Purified; Purified by Protein A affinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.

• Applicable Applications for anti-AFG3L2 antibody: ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: Suitable for use in Western Blot, Immunohistochemistry, ELISA; Dilution: ELISA: 1:1,000; Western Blot: 1:100-500; Immunohistochemistry: 1:10-50
• Immunogen: AFG3L2 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 51-80 amino acids from the N-terminal region of human AFG3L2.
• Preparation and Storage: May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(AFG3L2 Antibody (N-term) western blot analysis in MDA-MB453,MCF-7,Jurkat cell line lysates (35ug/lane).This demonstrates the AFG3L2 antibody detected the AFG3L2 protein (arrow).)

Immunohistochemistry (IHC)

(AFG3L2 Antibody (N-term) immunohistochemistry analysis in formalin fixed and paraffin embedded human cerebellum tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of AFG3L2 Antibody (N-term) for immunohistochemistry. Clinical relevance has not been evaluated.)

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

Antibodies; Abs to Proteins

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 ATPase family member 3-like 2 (S. cerevisiae)
• NCBI Official Symbol: AFG3L2
• NCBI Official Synonym Symbols: SCA28; SPAX5
• NCBI Protein Information: AFG3-like protein 2; paraplegin-like protein; ATPase family gene 3, yeast; AFG3 ATPase family gene 3-like 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: Mitochondrial; Chaperone; Protease; Membrane protein, multi-pass; EC 3.4.24.-; Membrane protein, integral; Cell development/differentiation

Chromosomal Location of Human Ortholog: 18p11

Cellular Component: mitochondrion; mitochondrial inner membrane; integral to membrane

Molecular Function: protein binding; zinc ion binding; metalloendopeptidase activity; unfolded protein binding; ATP binding

Biological Process: myelination; cristae formation; axonogenesis; mitochondrial fusion; righting reflex; death; regulation of multicellular organism growth; nerve development; proteolysis; muscle fiber development; neuromuscular junction development

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