Amyloid fibril protein nomenclature: 2010 recommendations from the nomenclature committee of the International Society of Amyloidosis

Figures & data

Table I. Amyloid fibril proteins and their precursors in humans.*

Amyloid protein	Precursor	Systemic (S) or localized, organ restricted (L)	Syndrome or involved tissues
AL	Immunoglobulin light chain	S, L	Primary
			Myeloma-associated
AH	Immunoglobulin heavy chain	S, L	Primary
			Myeloma-associated
Aβ2M	β2-microglobulin	S	Hemodialysis-associated
		L?	Joints
ATTR	Transthyretin	S	Familial
			Senile systemic
		L?	Tenosynovium
AA	(Apo)serum AA	S	Secondary, reactive
AApoAI	Apolipoprotein AI	S	Familial
		L	Aorta, meniscus
AApoAII	Apolipoprotein AII	S	Familial
AApoAIV	Apolipoprotein AIV	S	Sporadic, associated with aging
AGel	Gelsolin	S	Familial (Finnish)
ALys	Lysozyme	S	Familial
AFib	Fibrinogen α-chain	S	Familial
ACys	Cystatin C	S	Familial
ABri	ABriPP	S	Familial dementia, British
ALect2	Leukocyte chemotactic factor 2	S	Mainly kidney
ADan*	ADanPP	L	Familial dementia, Danish
Aβ	Aβ protein precursor (AβPP)	L	Alzheimer's disease, aging
APrP	Prion protein	L	Spongioform encephalopathies
ACal	(Pro)calcitonin	L	C-cell thyroid tumors
AIAPP	Islet amyloid polypeptide**	L	Islets of Langerhans
			Insulinomas
AANF	Atrial natriuretic factor	L	Cardiac atria
APro	Prolactin	L	Aging pituitary
			Prolactinomas
AIns	Insulin	L	Iatrogenic
AMed	Lactadherin	L	Senile aortic, media
AKer	Kerato-epithelin	L	Cornea, familial
ALac	Lactoferrin	L	Cornea
AOaap	Odontogenic ameloblast-associated protein	L	Odontogenic tumors
ASemI	Semenogelin I	L	Vesicula seminalis

*Proteins are listed, when possible, according to relationship. Thus, apolipoproteins are grouped together, as are polypeptide hormones.

^†ADan comes from the same gene as ABri.

^‡Also called ‘amylin’.

Table II. Amyloid fibril proteins and their precursors in animals.

Amyloid protein	Precursor	Systemic (S) or localized (L)	Syndrome or involved tissues	Species
AL	Immunoglobulin light chain	S, L	Plasmocytoma	Horse, cat
AA	(Apo)serum AA	S	Secondary, reactive	Mouse, guinea pig, cat, dog, cow, duck, etc
AApoAI	Apolipoprotein AI	S	Age-related	Dog
AApoAII	Apolipoprotein AII	S	Age-related	Mouse
ATTR	Transthyretin*	S	Age-related	Monkey
Aβ	Aβ protein precursor	L	Age-related	Dog, sheep, wolverine
AIAPP	Islet amyloid polypeptide	L	Islets of langerhans	Cats, apes, raccoon
			Insulinoma
AIns	Insulin	L	Islets of langerhans	Octodon degus
ACas	α-S2C casein	L	Mammary gland	Cow

*Provisional, awaiting DNA or protein sequence analysis.

Table III. Intracellular inclusions with known biochemical composition, with or without amyloid properties.

Inclusion name	Site	Protein nature	Examples of associated disease
Lewy bodies	Neurons	α-synuclein*	Parkinson's disease
	Intracytoplasmic
Huntington bodies	Neurons	PolyQ expanded huntingtin	Huntington's disease
	Intranuclear
Hirano bodies	Neurons	Actin	Neurodegenerative disorders
Collins bodies	Neurons	Neuroserpin	Forms of familial presenile dementia
Not specified	Neurons, many	Ferritin	Form of familial neurodegenerative disorder
	Different cells
Neurofibrillary tangles	Neurons	Tau	Alzheimer disease, fronto-temporal dementia, aging, other cerebral conditions
	Intracytoplasmic

*Simplified. Additional components may exist.