Anti-AFG3L2 항체 (CAB15393)

Antibody Name:	Anti-AFG3L2 Antibody
Antibody SKU:	CAB15393
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 538-797 of human AFG3L2 (NP_006787.2).

Application:	WB IHC IF
Recommended Dilution:	WB 1:200 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:	LO2, 293T, HeLa, Mouse brain, Mouse heart, Mouse kidney, Mouse liver, Mouse pancreas

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 538-797 of human AFG3L2 (NP_006787.2).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	DSIN QKHF EQAI ERVI GGLE KKTQ VLQP EEKK TVAY HEAG HAVA GWYL EHAD PLLK VSII PRGK GLGY AQYL PKEQ YLYT KEQL LDRM CMTL GGRV SEEI FFGR ITTG AQDD LRKV TQSA YAQI VQFG MNEK VGQI SFDL PRQG DMVL EKPY SEAT ARLI DDEV RILI NDAY KRTV ALLT EKKA DVEK VALL LLEK EVLD KNDM VELL GPRP FAEK STYE EFVE GTGS LDED TSLP EGLK DWNK EREK EKEE PPGE KVAN
Gene ID:	10939
Uniprot:	Q9Y4W6
Cellular Location:	Mitochondrion membrane, Multi-pass membrane protein
Calculated MW:	88kDa
Observed MW:	89kDa

Synonyms:	AFG3L2, SCA28, SPAX5
Background:	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.

UniProt Protein Function:	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.
UniProt Protein Details:	Protein type:Protease; EC 3.4.24.-; Chaperone; Membrane protein, multi-pass; Mitochondrial; Membrane protein, integral; Cell development/differentiation 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
NCBI Summary:	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 Code:	Q9Y4W6
NCBI GenInfo Identifier:	126302516
NCBI Gene ID:	10939
NCBI Accession:	Q9Y4W6.2
UniProt Secondary Accession:	Q9Y4W6,Q6P1L0,
UniProt Related Accession:	Q9Y4W6
Molecular Weight:	88,584 Da
NCBI Full Name:	AFG3-like protein 2
NCBI 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
UniProt Synonym Protein Names:	Paraplegin-like protein
Protein Family:	AFG3-like protein
UniProt Gene Name:	AFG3L2
UniProt Entry Name:	AFG32_HUMAN

	Western blot analysis of extracts of various cell lines, using AFG3L2 antibody (CAB15393) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 5s.
	Immunohistochemistry of paraffin-embedded rat brain using AFG3L2 antibody (CAB15393) at dilution of 1:200 (40x lens).
	Immunohistochemistry of paraffin-embedded human appendix using AFG3L2 antibody (CAB15393) at dilution of 1:200 (40x lens).

Antibodies

AFG3L2 Antibody (PACO07636)

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)